Category Archive Skin & Beauty Care

Swim Spa – Health Benefits of Swim Spas

A swim spa is a multifunctional machine that combines the best features of a swimming pool and a hot tub. It also tends to fall somewhere in the middle of these two in terms of size.

One of the most common ways that swim spas are used is for a challenging but impact-free water workout. Swim spas produce a powerful current of water that provides continuous resistance you can swim, run, jog, or walk against without ever “hitting the wall.” You can swim lap after lap, or walk or run for miles, all in the comfort and privacy of your swim spa.

Best of all, once you’ve given your muscles a good workout, you can use the very same swim spa to relax and unwind. Simply settle down in front of the hydrotherapy jets and adjust the settings for an invigorating and therapeutic massage. So, to sum up, a swim spa offers a range of activities geared toward fitness, fun, and relaxation:

  • Swim, run, jog, or walk against a powerful resistance-producing current
  • Splash around with your family and friends
  • Relax into an ergonomic seat surrounded by heated water
  • Soothe sore muscles with massage jets

What is a Swim Spa? The Basics

Swim spas are an alternative to traditional pools and spas. Like hot tubs, swim spas are self-contained, so all the pumps, filters and heating elements are part of the unit — no additional equipment required! However, like swimming pools, swim spas are designed with swimming in mind.

With powerful jets on one side, swim spas produce a swim current, allowing users to swim continuously in one place. When these jets are turned on, it creates an endless pool effect so swimmers can backstroke, butterfly or freestyle as long as they want.

Swim spas are a popular option for people who enjoy pool exercises and swimming workouts. However, most swim spas are split into two sections, with a pool on one side and a hot tub with jets and seating on the other end, so users can get a workout in, and then relax.

Don’t let the word “spa” fool you: the water temperature is adjustable to serve a wide range of uses. Additionally, there are several models and types. You can have an in-ground swim spa or an above-ground swim spa, and you can even choose an indoor or outdoor model.

8 Benefits of Swim Spas

Let’s move on to some of the most significant benefits of swim spas.

1. Less Space:

You don’t need a lot of room to swim laps with swim spas, so they are generally much smaller than traditional swimming pools.

2. Multi-Functional:

Most swim spas provide a space for continuous swimming exercise, hot tub relaxation and entertainment.

3. Year-Round Use:

Because the water is heated, swim spas are meant to be used throughout the entire year. (For this reason, most swim spa owners invest in an automatic pool cover, so they can easily keep their swim spa protected and accessible year-round).

4. Ideal Exercise Environment:

Aquatic exercises are low-impact and great for any fitness level, and swim spas provide space for just that. The buoyancy of water is an excellent choice for people with chronic pain to remain active, improve flexibility, increase balance and enjoy all the other well-known benefits of exercise.

5. Unparalleled Swim Training:

Whether you’re training for a triathlon or competitive swimming, swim training is essential. However, training in public pools, gyms and aquatic centers may not be an option. Swim spas allow swimmers to practice their crawl, backstroke, breaststroke, butterfly and more from the comfort of their backyard — just adjust the water temperature as you like and start swimming.

6. Warm Water & Aquatic Therapy:

You can easily heat up the water temperature of swim spas for therapeutic purposes. Combine this with a water-based aquatic therapy routine, and swim spas can help people struggling with chronic pain.

7. Relaxation:

Don’t forget about the spa part of the swim spa. Most swim spas include massaging jets and other features found in traditional hot tubs, so you can turn up the temperature and use your swim spa to ease tension and relax after a long day (or high-power swimming workout).

8. Entertainment:

Like any pool or spa, a swim spa is a great space to hang out with friends and family. Splash around or relax and catch up. Swim spas offer many of the same benefits of regular pools and spas.

Healthy Living with a Swim Spa

Many of our clients find that they naturally (and almost effortlessly) live a healthier lifestyle once they add a swim spa to their home.

Keeping up a fitness schedule no longer requires planning, commuting, dressing, and undressing in a locker room, and all the other small hassles that working out in a public facility—even a high-end one—requires. Going for a swim or a water run becomes a simple matter of changing into a swimsuit and hitting the water.

Another way that swim spas make adopting a healthier lifestyle a breeze is a fact that water workouts tend to be really fun and enjoyable. It’s so much easier to stick with an exercise regimen when it’s something you actually look forward to doing. And again, because it’s in the privacy of your home, you can create the ultimate personalized workout environment. Blast your favorite music, set out a stack of fluffy towels to dry off with, and get your favorite smoothie ready to reward yourself with after you step out of the pool.

Finally, swim spas help you live a healthier lifestyle by completely eliminating one huge obstacle that prevents many people from working out consistently: the shyness factor. It can be very intimidating to exercise in front of other people, especially if we’re trying something new. A swim spa offers you complete privacy, and if you don’t already know how to swim or do water aerobics, you can take lessons from a private coach in a comfortable, one-on-one setting in your home.

How a Swim Spa Can Help

Better health and well-being don’t come from a single choice or action—it’s typically the result of our habits. If we want to enjoy the fruits of a strong body and mind, then it’s in our interest to create a lifestyle that supports the development of good habits.

A swim spa is an excellent way to develop a routine. It’s hard to think of a better or more convenient way to get a full-body workout than in the comfort and privacy of your own swim spa. You can use it as often as you like, and in a variety of different ways, to achieve your health and wellness goals.

In terms of physical health, a swim spa offers the following benefits:

  • Eases muscle tension
  • Soothes joint pain
  • Builds a stronger cardiovascular system
  • Promotes weight loss
  • Increases muscle strength
  • Improves circulation

A swim spa is able to produce such a wide range of positive health outcomes because it provides the perfect setting for hydrotherapy.

Hydrotherapy is a water-based therapy that has been used for decades all around the world as a way to heal injuries, improve quality of life, and promote overall well-being. It draws on the healing qualities of water—namely, its temperature and buoyancy—in combination with massage to boost blood circulation and the production of endorphins.

Speaking of endorphins, which are the body’s “feel-good” hormones, the health benefits of a swim spa don’t stop at just the physical level.

A swim spa also offers a range of mental health benefits as well:

  • Improves sleeps patterns
  • Reduce stress and anxiety
  • Promotes relaxation
  • Calms the mind

This is because swim spas are a supremely relaxing environment that promotes mental calmness and clarity. Sinking into the heated waters and letting your stress melt away under the gentle pressure of the massage jets is a deeply healing experience that carries over into the rest of your life. The enhanced sense of well-being that you have after spending time in your swim spa helps you regulate your sleep patterns so you can fall asleep and stay asleep for a good night’s rest, which in turn, leads to an increased ability to handle the stresses of daily life.

Other Benefits of Owning a Swim Spa

Part of health and well-being is feeling good about our choices and enjoying the life we have created for ourselves. On this point, too, swim spas are an excellent way to invest in yourself, your home, and your family.

Enjoy more quality family time

With the fast pace of modern life, It can be difficult to find time to spend together as a family. Having a swim spa right at home provides the perfect gathering place for you and your family members to get together, enjoy each other’s company, and catch up on each other’s lives. Family swim time may just become the highlight of your week.

Upgrade your backyard

There’s no doubt about it—a swim spa is a stunning addition to a backyard and is sure to draw the attention and admiration of your neighbors. You may even inspire some to get their own swim spas!

Increase the value of your home

Like any renovation or extension to your home, a swim spa adds value to your property. It is a unique and attractive feature that will not only make your property stand out if and when it ever goes on the market, but it will also attract higher bids because of it.

Provide an excellent venue for hosting gatherings

Having a strong social network is another important element in living a healthier life. A swim spa gives you a great excuse to get together more often. Throw a mini-pool party for your closest friends, or just have one or two of your favourite people over to unwind with the massage jets and watch the sun go down.

Allow you to spend more time outdoors

Most of us would like to spend more time outdoors, and a backyard swim spa lets you do just that. Because a swim spa uses heated water, it’s a feature you can use 365 days of the year, through every season. Breezy spring days, bright summer afternoons, crisp fall evenings, and calm winter nights are all excellent times to enjoy a soak.

More Ways to Live a Healthier Lifestyle

Once you’ve felt the increased energy, improved mood, and overall boost in well-being that comes from taking good care of yourself, you’ll want to keep it going by making more positive changes in your life. Here are a few ideas to keep you on track for a healthier lifestyle.

Limit your screen time

Spending too much time-consuming media is bad for us. Most of us don’t need statistics to prove it (although there are plenty of those) because we know how we feel after binging on too many TV shows or staying up too late scrolling on our phones. Making a conscious effort to limit your screen time is an effective way to improve your mental health. If you need help doing this, you can adjust your smartphone’s settings to track your screen time and install apps that automatically close down your apps after a certain amount of time.

Try new hobbies

Trying new things is a time-tested way to build your confidence and reignite your zest for life. Let your own personal interests guide you to a new hobby or pastime, whether it’s photography, painting, sewing, or yoga (which you can also do in your swim spa!).

Eat a healthy, well-balanced diet

Along with exercise, the food we eat is one of the biggest determiners of our health and well-being. A well-balanced diet is one that includes a generous amount of fruits, vegetables, grains, and lean proteins, and avoids highly processed foods with high amounts of sugar and fat. Many people find that once they start exercising, they naturally feel motivated to eat well, since quality food energizes them and powers their workouts.

Read more books

Reading is an amazing way to broaden your horizons and stimulate your intellect. Whether you prefer fiction, non-fiction, or both, there’s an entire world of knowledge and creativity waiting for you when you pick up a book. Consider joining a book club in order to enjoy another layer of health benefits from connecting with others and expanding your social world.

Grow a garden

Bursting with lush green life, gardens are naturally relaxing environments. Growing a garden in your backyard will not only create a beautiful natural oasis, it also helps support local bug and bird populations (especially if you choose to grow plants that are native to your area). What’s more, a healthy garden improves the air quality of the surrounding area and provides stunning natural scenery to enjoy from the relaxing comfort of your swim spa.

Practice meditation

Meditation is an ancient practice that has exploded in popularity all over the world today, with good reason. The health benefits of meditation are extensive, from improving mental clarity to reducing high blood pressure. One of the best things about meditation is that you can do it pretty much anywhere and anytime. All you have to do is find a quiet space, focus on your breath, and clear your mind.

What’s the Best Swim Spa for Me?

Ultimately, the best swim spa for you comes down to personal preference. You can have one custom-built or order a prefabricated swim spa. There are so many options that it’s best to look around a bit to see which features you want and need most.

Once you decide on a model, be sure to add an automatic pool cover for your swim spa to keep it protected throughout the year. Plus, an auto pool cover can help you control operational costs by trapping heat.

 

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Polycystic Kidney Disease – Causes, Symptoms, Treatment

Polycystic kidney disease is a disorder that affects the kidneys and other organs. Clusters of fluid-filled sacs, called cysts, develop in the kidneys and interfere with their ability to filter waste products from the blood. The growth of cysts causes the kidneys to become enlarged and can lead to kidney failure.

Polycystic kidney disease (ADPKD) is an autosomal dominant disorder. It is a multisystem and progressive disease with cysts formation and kidney enlargement along with other organ involvement (e.g., liver, pancreas, spleen). This activity describes the evaluation and management of polycystic kidney disease and highlights the role of the interprofessional team in improving care for patients with this condition.

Polycystic kidney disease (ADPKD) is an autosomal dominant disorder. It’s a multisystem and progressive disease with cysts formation and kidney enlargement along with other organ involvement (e.g., liver, pancreas, spleen).

Causes of Polycystic Kidney Disease

ADPKD is an autosomal dominant disease, so it is found in males and females equally, and each offspring has a 50% chance of inheriting the disease.

ADPKD involves at least two genes. PKD1 accounts for most ADPKD cases and is located on 16p13.3. PKD2 accounts for 15% of ADPKD cases.

PKD1 codes for polycystin 1, a 4304 amino acid protein. Polycystin 1 interacts with polycystin 2 and is involved in cell cycle regulation and intracellular calcium transport. PKD2 codes for polycystin two, which is structurally similar to polycystin 1. It is a member of the family of voltage-activated calcium channels.

Polycystin 1 and two are located in the epithelial cells of the renal tubules and other areas of the renal cell epithelium. Both form heteromeric complexes and are found in the primary cilium of epithelial cells in kidneys. The primary cilium is considered a mechanical receptor that can sense changes in tubular fluid flow, and that can transduce them into intracellular calcium signaling. ADPKD1 is more severe than ADPKD2.

Mutations in the PKD1PKD2, and PKHD1 genes cause polycystic kidney disease.

Mutations in either the PKD1 or PKD2 gene can cause autosomal dominant polycystic kidney disease; PKD1 gene mutations cause ADPKD type 1, and PKD2 gene mutations cause ADPKD type 2. These genes provide instructions for making proteins whose functions are not fully understood. Researchers believe that they are involved in transmitting chemical signals from outside the cell to the cell’s nucleus. The two proteins work together to promote normal kidney development, organization, and function. Mutations in the PKD1 or PKD2 gene lead to the formation of thousands of cysts, which disrupt the normal functions of the kidneys and other organs. People with mutations in the PKD2 gene, particularly women, typically have a less severe form of the disease than people with PKD1 mutations. The signs and symptoms, including a decline in kidney function, tend to appear later in adulthood in people with a PKD2 mutation.

Mutations in the PKHD1 gene cause autosomal recessive polycystic kidney disease. This gene provides instructions for making a protein whose exact function is unknown; however, the protein likely transmits chemical signals from outside the cell to the cell nucleus. Researchers have not determined how mutations in the PKHD1 gene lead to the formation of numerous cysts characteristic of polycystic kidney disease.

Although polycystic kidney disease is usually a genetic disorder, a small percentage of cases are not caused by gene mutations. These cases are called acquired polycystic kidney disease. This form of the disorder occurs most often in people with other types of kidney disease who have been treated for several years with hemodialysis (a procedure that filters waste products from the blood).

Polycystin-1 (PKD1 protein) and polycystin-2 (PKD2 protein) belong to a subfamily of transient receptor potential (TRP) channels. PKD2 is found in the endoplasmic reticulum and also in the plasma membrane in spindles in cell division and primary cilium. PKD1 inactivation determines the rate of development of the cystic disease. PC1 interacts with PC2 and helps in the maturation of PC2, and vice versa. PC1 and PC2 also interact with additional calcium channel proteins.

A common finding in animal models of PKD has increased levels of cyclic adenosine monophosphate (cAMP), not only in the kidney but also in the liver and vascular smooth muscle. cAMP exerts effects on cell proliferation in different cell types. cAMP and PKA signaling enhances several pro-proliferative pathways in cells derived from polycystic kidneys while inhibiting proliferation in cells derived from the normal human kidney cortex.

Liver cysts arise by excessive proliferation and dilation of biliary ductules and peribiliary glands.

Endothelial vasodilation and constitutive nitric oxide synthase activity are reduced in subcutaneous resistance vessels from patients with ADPKD and normal glomerular filtration rate (GFR), thus causing hypertension.

Symptoms of Polycystic Kidney Disease

Polycystic kidney disease symptoms can include:

  • High blood pressure
  • Back or side pain
  • Blood in your urine
  • A feeling of fullness in your abdomen
  • Increased size of your abdomen due to enlarged kidneys
  • Headaches
  • Kidney stones
  • Kidney failure
  • Urinary tract or kidney infections

Diagnosis of Polycystic Kidney Disease

History and Physical

Renal size increases with age, and renal enlargement eventually occurs in 100% of patients with ADPKD. The severity of the structural abnormality correlates with the manifestations of ADPKD, such as pain, hematuria, hypertension, and renal impairment. Most clinical manifestations are directly related to the enlargement of renal cysts. 

Episodes of acute renal pain are seen quite often due to cyst hemorrhage, infection, stone, and, rarely, tumors. Visible hematuria may be the initial presenting symptom. Cyst hemorrhage is a frequent complication causing gross hematuria when the cyst communicates with the collecting system. It can manifest with fever, raising the possibility of cyst infection. Occasionally a hemorrhagic cyst will rupture, resulting in a retroperitoneal bleed.

Urinary tract infection (UTI) is common in ADPKD. UTI presents as cystitis, acute pyelonephritis, cyst infection, and perinephric abscesses. Most infections are caused by Escherichia coli, Klebsiella and Proteus species, and other Enterobacteriaceae.

Both CT and MRI are sensitive to detect complicated cysts.

Renal stone disease occurs in about 20% of patients with ADPKD. Most stones are composed of uric acid, calcium oxalate, or both. Stones can be difficult to diagnose on imaging in ADPKD because of cyst wall and parenchymal calcification. CT urography is helpful.

Hypertension is the most common manifestation of ADPKD. Microalbuminuria, proteinuria, and hematuria are more common in hypertensive patients with ADPKD.

In a majority of patients, renal function is within the normal range. By the time renal function starts worsening, the kidneys usually are greatly enlarged. ESRD is not inevitable in ADPKD. Up to 77% of patients are alive with preserved renal function at age 50 years, and 52% at age 73. Kidney and cyst volumes are the strongest predictors of renal functional decline.

Most simple hepatic cysts are solitary, and PLD should be suspected when four or more cysts are present in the hepatic parenchyma.

Lab Test and Imaging

Ultrasound criteria for the diagnosis of ADPKD:

Original Ravine’s PKD1 Diagnostic Criteria

  • Age 15-29 years – two or more cysts unilateral or bilateral
  • Age 30-39 years – two or more cysts in each kidney
  • Age 40-49 years – two or more cysts in each kidney
  • Age 60 years or above – four or more cysts in each kidney

Genetic testing is done when a precise diagnosis is not confirmed enlargement along, and the results of imaging are indeterminate.

Treatment of Polycystic Kidney Disease

  • Flank Pain – Causes of flank pain that may require intervention, such as infection, stone, and tumor, should be excluded. Opioid analgesics should be reserved for the management of acute pain. Reassurance, lifestyle modification, and avoidance of aggravating activities may be helpful. Tricyclic antidepressants are helpful as in other chronic pain syndromes, as they are well tolerated. Cyst aspiration, under ultrasound or CT guidance, can be done if distortion of the kidney by a large cyst is considered the cause of the pain. If multiple cysts are contributing to pain, laparoscopic or surgical cyst fenestration may be of benefit.
  • Cyst hemorrhage – Cyst hemorrhage episodes are self-limited, and patients respond well to conservative management with bed rest, analgesics, and increased fluid intake to prevent obstructing clots. Rarely, bleeding is more severe, leading to hemodynamic instability; this requires hospitalization and transfusions.
  • Cyst and urinary tract infection – Immediate treatment of symptomatic cystitis and asymptomatic bacteriuria is indicated to prevent retrograde seeding of the renal parenchyma. Agents of choice include trimethoprim-sulfamethoxazole and fluoroquinolones. If fever persists after 1 to 2 weeks of appropriate antimicrobial therapy, infected cysts should be drained percutaneously or surgically. In the case of end-stage polycystic kidneys, nephrectomy should be considered.
  • Nephrolithiasis – Potassium citrate is the treatment of choice in stone-forming conditions associated with it, which are uric acid stones, hypo-citraturic calcium oxalate stones, and distal acidification disorders.
  • Hypertension – Angiotensin-converting enzyme (ACE) inhibitors or angiotensin receptor blockers (ARBs) increase renal blood flow in ADPKD and are antihypertensives of choice.

Treating polycystic kidney disease involves dealing with the following signs, symptoms, and complications in their early stages:

  • Kidney cyst growth – Tolvaptan therapy may be recommended for adults at risk of rapidly progressive ADPKD. Tolvaptan (Jynarque, Samsca) is a pill that you take by mouth that works to slow the rate of kidney cyst growth and the decline in how well your kidneys work. There’s a risk of serious liver injury when taking tolvaptan, and it can interact with other medicines you take. It’s best to see a doctor who specializes in kidney health (nephrologist) when taking tolvaptan so that you can be monitored for side effects and possible complications.
  • High blood pressure – Controlling high blood pressure can delay the progression of the disease and slow further kidney damage. Combining a low-sodium, low-fat diet that’s moderate in protein and calorie content with not smoking, increasing exercise, and reducing stress may help control high blood pressure. However, medications are usually needed to control high blood pressure. Medications called angiotensin-converting enzyme (ACE) inhibitors or angiotensin II receptor blockers (ARBs) are often used to control high blood pressure.
  • Declining kidney function. To help your kidneys stay as healthy as possible for as long as possible, experts recommend maintaining a normal body weight (body mass index). Drinking water and fluids throughout the day may help slow the growth of kidney cysts, which in turn could slow down a decline in kidney function. Following a low-salt diet and eating less protein might allow kidney cysts to respond better to the increase in fluids.
  • Pain. You might be able to control the pain of polycystic kidney disease with over-the-counter medications containing acetaminophen. For some people, however, the pain is more severe and constant. Your doctor might recommend a procedure using a needle to draw out cyst fluid and inject a medication (sclerosing agent) to shrink kidney cysts. Or you may need surgery to remove cysts if they’re large enough to cause pressure and pain.
  • Bladder or kidney infections. Prompt treatment of infections with antibiotics is necessary to prevent kidney damage. Your doctor may investigate whether you have a simple bladder infection or a more complicated cyst or a kidney infection. For more complicated infections, you may need to take a longer course of antibiotics.
  • Blood in the urine. You’ll need to drink lots of fluids, preferably plain water, as soon as you notice blood in your urine to dilute the urine. Dilution might help prevent obstructive clots from forming in your urinary tract. In most cases, the bleeding will stop on its own. If it doesn’t, it’s important to contact your doctor.
  • Kidney failure. If your kidneys lose their ability to remove waste products and extra fluids from your blood, you’ll eventually need either dialysis or a kidney transplant. Seeing your doctor regularly for monitoring of PKD allows for the best timing of a kidney transplant. You may be able to have a preemptive kidney transplant, which means you wouldn’t need to start dialysis but would have the transplant instead.
  • Aneurysms. If you have polycystic kidney disease and a family history of ruptured brain (intracranial) aneurysms, your doctor may recommend regular screening for intracranial aneurysms.

If an aneurysm is discovered, surgical clipping of the aneurysm to reduce the risk of bleeding may be an option, depending on its size. Nonsurgical treatment of small aneurysms may involve controlling high blood pressure and high blood cholesterol, as well as quitting smoking.

Should people with PKD take a special diet?

  • At present, no specific diet is known to prevent cysts from developing in patients with PKD. Reducing salt intake helps control blood pressure in PKD patients who have high blood pressure. A diet low in fat and moderate in calories is recommended to maintain a healthy weight. Speak to your doctor or a dietitian about other changes to your diet, such as avoiding caffeine.

Is exercise recommended for people with PKD?

  • Absolutely. However, exercises that are potentially harmful to the kidney, such as contact sports, should be avoided. It is important not to become too dehydrated during any physical activity.

Who is at risk for developing PKD?

PKD runs in families. It is an inherited disorder that is passed from parents to children through genes. Genes are the basic elements of heredity. At conception, children receive a set of genes from each parent. They determine many characteristics such as hair color and eye color. Genes can also determine the likelihood of developing a disease.

A genetic disease can happen if one or both parents pass abnormal genes to a child. This happens through something called dominant inheritance or recessive inheritance.

  • Dominant inheritance – If one parent has the disease and passes an abnormal gene to the child, it is called dominant inheritance. Each child has a 50% chance of getting the disease. The risk is the same for every child, regardless of how many children develop the disease.
  • Recessive inheritance – If both parents carry the abnormal gene, and both parents pass an abnormal gene to the child, it is called recessive inheritance. In this situation, every child has a 25% chance of getting the disease.

Are there different types of PKD?

Yes. The three main types of PKD are

    • Autosomal Dominant PKD – (also called PKD or ADPKD)This form of the disease is passed from parent to child by dominant inheritance. In other words, only one copy of the abnormal gene is needed to cause the disease. Symptoms usually begin between the ages of 30 and 40, but they can begin earlier, even in childhood. ADPKD is the most common form of PKD. In fact, about 90 percent of all PKD cases are ADPKD.
    • Infantile or Autosomal Recessive PKD(also called ARPKD) – This form of the disease is passed from parent to child by recessive inheritance. Symptoms can begin in the earliest months of life, even in the womb. It tends to be very serious, progresses rapidly, and is often fatal in the first few months of life. This form of ARPKD is extremely rare. It occurs in 1 out of 25,000 people.
    • Acquired Cystic Kidney Disease (also called ACKD) – ACKD can happen in kidneys with long-term damage and severe scarring, so it is often associated with kidney failure and dialysis. About 90 percent of people on dialysis for 5 years develop ACKD. People with ACKD usually seek help because they notice blood in their urine. This is because the cysts bleed into the urinary system, which discolors urine.

Complications

Complications associated with polycystic kidney disease include:

  • High blood pressure – Elevated blood pressure is a common complication of polycystic kidney disease. Untreated, high blood pressure can cause further damage to your kidneys and increase your risk of heart disease and strokes.
  • Loss of kidney function – Progressive loss of kidney function is one of the most serious complications of polycystic kidney disease. Nearly half of those with the disease have kidney failure by age 60. PKD can interfere with the ability of your kidneys to keep wastes from building to toxic levels, a condition called uremia. As the disease worsens, end-stage kidney (renal) disease may result, necessitating ongoing kidney dialysis or a transplant to prolong your life.
  • Chronic pain – Pain is a common symptom for people with polycystic kidney disease. It often occurs in your side or back. The pain can also be associated with a urinary tract infection, a kidney stone or a malignancy.
  • Growth of cysts in the liver – The likelihood of developing liver cysts for someone with polycystic kidney disease increases with age. While both men and women develop cysts, women often develop larger cysts. Female hormones and multiple pregnancies might contribute to liver cyst development.
  • Development of an aneurysm in the brain – A balloon-like bulge in a blood vessel (aneurysm) in your brain can cause bleeding (hemorrhage) if it ruptures. People with polycystic kidney disease have a higher risk of aneurysms. People with a family history of aneurysms seem to be at the highest risk. Ask your doctor if screening is needed in your case. If screening reveals that you don’t have an aneurysm, your doctor may recommend repeating the screening exam in a few years or after several years as a follow-up. The timing of repeat screening depends on your risk.
  • Pregnancy complications – Pregnancy is successful for most women with polycystic kidney disease. In some cases, however, women may develop a life-threatening disorder called preeclampsia. Those most at risk have high blood pressure or a decline in kidney function before they become pregnant.
  • Heart valve abnormalities – As many as 1 in 4 adults with polycystic kidney disease develops mitral valve prolapse. When this happens, the heart valve no longer closes properly, which allows blood to leak backward.
  • Colon problems –  Weaknesses and pouches or sacs in the wall of the colon (diverticulosis) may develop in people with polycystic kidney disease.

Make lifestyle changes

  • Be active for 30 minutes or more on most days. Regular physical activity can help you reduce stress, manage your weight, and control your blood pressure. If you are not active now, ask your health care provider about how much and what type of physical activity is right for you. If you play contact sports, such as football or hockey, a health care provider should do a magnetic resonance imaging (MRI) test to see whether these sports are safe for you. Trauma to your body, especially to your back and sides, may cause kidney cysts to burst.
  • Lose weight. Being overweight makes your kidneys work harder. Losing weight helps protect your kidneys.
  • Aim for 7 to 8 hours of sleep each night. Getting enough sleep is important to your overall physical and mental health and can help you manage your blood pressure and blood glucose, or blood sugar.
  • Reduce stress. Long-term stress can raise your blood pressure and even lead to depression. Some of the steps you take to manage your PKD are also healthy ways to cope with stress. For example, getting enough physical activity and sleep helps reduce stress.
  • Quit smoking. Cigarette smoking can raise your blood pressure, making your kidney damage worse. Quitting smoking may help you meet your blood pressure goals, which is good for your kidneys and can lower your chances of having a heart attack or stroke. Quitting smoking is even more important for people with PKD who have aneurysms. An aneurysm is a bulge in the wall of a blood vessel.

Prevention

If you have polycystic kidney disease and you’re considering having children, a genetic counselor can help you assess your risk of passing the disease to your offspring.

Keeping your kidneys as healthy as possible may help prevent some of the complications of this disease. One of the most important ways you can protect your kidneys is by managing your blood pressure.

Here are some tips for keeping your blood pressure in check:

  • Take the blood pressure medications prescribed by your doctor as directed.
  • Eat a low-salt diet containing plenty of fruits, vegetables, and whole grains.
  • Maintain a healthy weight. Ask your doctor what the right weight is for you.
  • If you smoke, quit.
  • Exercise regularly. Aim for at least 30 minutes of moderate physical activity most days of the week.
  • Limit alcohol use.

References

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Angioedema – Causes, Symptoms, Diagnosis, Treatment

Angioedema is non-pitting edema that involves subcutaneous and/or submucosal layers of tissue that affect the face, lips, neck, and extremities, oral cavity, larynx, and/or gut. It becomes life-threatening when it involves the larynx. This activity examines the differential diagnosis of this condition and how to properly evaluate a patient presenting with it. This activity highlights the role of the interprofessional team in caring for patients with this condition.

Angioedema is characterized by one or more areas of well-demarcated, non-pitting edema of deep subcutaneous tissues. It is primarily known to involve the face, lips, tongue, and oropharynx, but also, can also involve the genitals, distal extremities, and gastrointestinal mucosa. Immediate identification of angioedema is of the utmost importance due to the potential for rapid development and progression of life-threatening airway compromise. This activity reviews the evaluation and treatment of angioedema and highlights the importance of a cohesive interprofessional team in providing optimal care to patients with angioedema.

Angioedema is a skin reaction similar to urticaria. It is most often characterized by an abrupt and short-lived swelling of the skin and mucous membranes. All parts of the body may be affected but swelling most often occurs around the eyes and lips. In severe cases, the internal lining of the upper respiratory tract and intestines may also be affected.

Angioedema is defined as ” subcutaneous tissues and/or submucosal tissues circumscribed non-pitting edema affecting lips, face, neck, and extremities oral cavity, larynx, and gut.” It becomes life-threatening when it involves the larynx, while intestinal angioedema is painful and mimics the acute abdomen.

Classification of angioedema

Acquired

  • Allergic (histaminergic angioedema) associated with anaphylaxis
  • Non-allergic (non-histaminergic angioedema), presenting isolated or in combination with urticaria
  • Drug-induced, e.g., angiotensin-converting enzyme inhibitors and non-steroidal anti-inflammatory drugs
  • Complement-mediated secondary to acquired deficiency of C1-inhibitor
  • Idiopathic which is subdivided into histaminergic and non-histaminergic

Hereditary forms

  • C1-Inhibitor deficiency divided into type 1 (lack of C1-inhibitor molecule) and typed 2 (dysfunctional C1-inhibitor molecule) with normal C1 inhibitor.

Causes of Angioedema

  • Inherited (hereditary angioedema) – Mutations in the gene encoding for C1-inhibitor cause hereditary angioedema and it is an autosomal dominant condition.
  • Acquired –  lymphoproliferative disorders, autoimmune, neoplastic, infection and drug-induced.
  • Histamine-mediated angioedema –  is the most common and is secondary to mast-cells and basophil activation.
  • Bradykinin-mediated angioedema – (hereditary angioedema, acquired C1-inhibitor deficiency and angiotensin-converting enzyme inhibitor-associated angioedema). Allergic reactions and hives do not trigger this condition. C1-inhibitor is a regulator of complement and the contact system; if deficient or dysfunctional it causes activation of the contact system resulting in uncontrolled production of kallikrein leading to proteolysis of high-molecular-weight kininogen and bradykinin, leading to edema by increasing in vascular permeability.
  • C1 inhibitor deficiency – Excessive production of bradykinin
  • Angiotensin-converting enzyme inhibitor-associated angioedema – Decreased degradation of bradykinin
  • Histamine and bradykinin – increase localized microvascular permeability.
  • NSAID induced – cyclooxygenase 1 inhibitor affects arachnoid acid metabolism, leukotriene/prostaglandin binding to the receptor or may be IgE mediated
  • Genetics – Hereditary angioedema can be secondary to the F12 gene, angiopoietin-1, and plasminogen or some unknown gene mutation.C1 inhibitor is a serine protease inhibitor (SERPIN)  C1 inhibitor deficiency is associated with  SERPING 1 mutation or mutated genes which encode for metabolizing and functioning enzymes of bradykinin. Abnormal accumulation of C1 inhibitor in dominant harmful disease affects plasma levels of hereditary angioedema type 1.
  • There are several etiologies of angioedema – most notably angiotensin-converting enzyme inhibitor (ACEI) use. With tens of millions of people taking ACEIs, the incidence of ACEI angioedema is rising. African Americans are at a much higher risk for ACEI angioedema, possibly due to lower endogenous bradykinin and increased sensitivity to ACEI-mediated increases in bradykinin.
  • Other etiologies include IgE-mediated allergic reactions – (such as to food, drug, or environmental triggers), nonsteroidal anti-inflammatory drug (NSAID) use (including aspirin), and chemically-induced histamine release (most commonly opiates, highly cationic antibiotics, and muscle relaxants). A less common cause is hereditary angioedema (HAE) and acquired angioedema (AAE), both caused by a C1-inhibitor (C1-INH) deficiency.
  • HAE and AAE are rare. AAE – is due to an acquired deficiency of (C1-INH), caused by either consumption (type 1) or inactivation (type 2). This increased catabolism can be related to an autoimmune disorder (e.g., systemic lupus erythematosus) or a malignant tumor (e.g., lymphoma). Angioedema recurs unpredictably, lasting from two to five days. It not only presents with edema of the areas mentioned above, but it can also cause severe abdominal pain due to gastrointestinal mucosa edema.

The causes of angioedema depend on the type of angioedema a patient has. Angioedema can be classified into at least four types, acute allergic angioedema, non-allergic drug reactions, idiopathic angioedema, hereditary angioedema (HAE) and acquired C1 inhibitor deficiency.

Angioedema type Causes
Acute allergic angioedema
(almost always occurs with urticaria within 1-2 hours of exposure to the allergen)
  • Food allergy, especially nuts, shellfish, milk, eggs
  • Drug-induced urticaria, eg, to penicillin, nonsteroidal anti-inflammatory drugs (NSAIDs), sulfa drugs, vaccines
  • Radiocontrast media
  • Insect venoms
  • Natural rubber latex, eg, medicinal gloves, catheters, balloons, contraceptive devices
Non-allergic drug reaction
(onset may be days to months after first taking the medication)
  • Angiotensin-converting enzyme (ACE) inhibitors
  • Cascade of effects via kinin production, arachidonic acid metabolism and nitric oxide generation
Idiopathic angioedema
(frequently chronic and relapsing and usually occurs with urticaria)
  • In most cases the cause of angioedema is unknown
  • Recent research indicates that 30–50% of this type of angioedema may be associated with some types of autoimmune disorders including systemic lupus erythematosus (SLE)
Hereditary angioedema
(very rare autosomal dominant inherited disease)
  • Inherited abnormal gene that causes a deficiency of a normal blood protein
  • 3 types: Type 1 and II mutations of C1NH (SERPING1) gene on chromosome 11, encoding C1 inhibitor protein; Type III mutation in F12 gene on chromosome 12, encoding coagulation factor XII.
  • Type 1 results in low levels and function of circulating C1 inhibitor; Type II has normal levels of C1 inhibitor protein but a reduction in function
  • Occurs in 1 in 50,000 males and females; Type III more severe in women.
  • Decreased C1 inhibitor activity leads to excessive kallikrein, which in turn produces bradykinin, a potent vasodilator
Acquired C1 inhibitor deficiency
  • Acquired during life rather than inherited
  • It May be due to B-cell lymphoma or antibodies against C1 inhibitor
Vibratory angioedema
  • A form of chronic inducible urticaria
  • Localized vibratory urticaria is also due to a vibratory stimulus and is considered distinct from vibratory angioedema
AAE recurrence is associated with various conditions such as different forms of lymphoproliferative disorders.
  • Acute contact dermatitis
  • Drug rash with eosinophilia and systemic symptoms
  • Dermatomyositis
  • Morbus Morbihan
  • Superior vena cava syndrome
  • Hypothyroidism
  • Subcutaneous emphysema
  • Orofacial granulomatosis
  • Hypocomplementemic urticarial vasculitis syndrome
  • Clarkson’s disease
  • Gleich’s syndrome
  • A cluster headache
  • Idiopathic edema 

Angioedema is one of the differential diagnoses in sudden onset of diffuse isolated edema.C1 inhibitor hereditary angioedema can be misdiagnosed as familial Mediterranean fever.

Symptoms of Angioedema

Symptoms and signs of angioedema may vary slightly between the different types of angioedema but in general, some or all of the following occur.

  • Marked swelling, usually around the eyes and mouth
  • Throat, tongue, hands, feet and/or genitals may be affected too
  • The skin may appear normal, i.e. no hives or another rash
  • Swellings may or may not be itchy
  • Swellings may be painful, tender or burning
  • In severe angioedema swelling of the throat and/or tongue may make it difficult to breath
  • Swelling of the lining of the intestinal tracts may cause gastrointestinal pain and cramps

Some features specific to the different types of angioedema are listed below.

Angioedema type Clinical features
Acute allergic angioedema
  • Almost always occurs with urticaria
  • Angioedema and urticaria both usually occur within 1-2 hours of exposure to an allergen (exception is ACE inhibitor-induced angioedema that usually occurs within the first week of treatment but can occur weeks to months later)
  • Reactions are usually self-limiting and subside within 1-3 days
  • Reactions will recur with repetitive exposures or exposure to cross-reactive substances
Non-allergic drug reaction
  • ACE inhibitor-induced angioedema occurs without urticaria
Idiopathic/chronic angioedema
  • Similar to acute allergic but angioedema keeps on recurring and often no known cause is found
Hereditary angioedema
  • Patients often experience no symptoms until they reach puberty
  • Swellings can occur without any provocation or induced by precipitating factors, including local trauma, vigorous exercise, emotional stress, alcohol, and hormonal factors (menstruation, pregnancy, estrogen)
  • Some patients may get a transitory prodromal non-itchy rash, headache, visual disturbance or anxiety
  • Face, hands, arms, legs, genitals, digestive tract, and airway may be affected; swellings spread slowly and may last for 3-4 days
  • Abdominal cramps, nausea, vomiting, difficulty breathing, and rarely urinary retention from swelling of internal tracts
  • Urticaria (wealing) does not occur
  • The tendency to angioedema is less pronounced in adults

Swellings that can look like angioedema

  • Contact allergy – from animals or plants is usually localized to the site of direct contact. It is often itchy, short-lived, does not cause internal swelling, and causes blistering rashes that weep and peel after a few days.
  • Animal allergy – can cause itchy hives (urticaria) and angioedema, but occurs mostly with severe hay fever (allergic rhinitis) and/or asthma.
  • Insect stings  – from bees, wasps, and some ants can cause severe local and temporary swelling.
  • Palindromic rheumatoid arthritis – is a rare form of arthritis that causes swellings that last a few days at a time, mainly over joints and affecting the limbs. Swellings are usually painful and hot rather than itchy. Some people with this condition will eventually go on to develop rheumatoid arthritis.

Less common mimics of angioedema – include the following causes of swelling that tend to persist:

  • Dermatomyositis (muscle weakness, facial redness, and swelling).
  • Blockage of the superior vena cava (a major vein in the chest that can result in fixed fluid accumulation in the neck and face and arms).
  • Underactive thyroid gland (which can cause puffiness of the face and lips known as myxoedema).
  • Facial rosacea (causing non-specific puffiness of the face, redness, flushing, and pimples).
  • Orofacial granulomatosis (fixed facial and lip swelling sometimes associated with inflammation of the bowel).
  • Subcutaneous emphysema (leakage of air into the soft tissues, often occurring after trauma or surgery).
  • Cluster headache (severe one-sided headache associated with puffiness around the eye on the same side).

Diagnosis of Angioedema

History and Physical

The presentation can be acute or chronic. Vital signs, level of consciousness, as well as a thorough ski, head, neck, respiratory, and abdomen evaluation should be completed.General monitoring of angioedema in the Emergency room includes Oxygen saturation and Cardiac status.

Histaminergic angioedema

Symptoms can involve different systems including skin (Urticaria, flushing, pruritus), Respiratory (bronchospasm), GI symptoms (abdominal pain and vomiting).  Onset is within 60 minutes of allergen exposure and may last for one to two days.

Etiologies: Drugs, foods, latex, and insect stings.

While comparing the bradykinin-mediated angioedema with histaminergic angioedema, the former has the following characteristics:

  • It is not associated with urticaria
  • More severe and longer duration
  • Has associated abdominal symptoms 

Lab Test

  • Acquired angioedema related to angiotensin-converting enzyme (ACE) inhibitor – It clinically presents as angioedema without urticaria or itching It is common in African-Americans. It can develop anytime but common in the first week of exposure.
  • Nonsteroidal anti-inflammatory disease-induced angioedema – NSAID-induced drug reactions include angioedema presenting with urticaria and facial swelling.
  • Hereditary angioedema – begins in childhood or young adulthood, gets worse at puberty and presents as recurrent episodes of swelling or abdominal pain. Patients can develop prominent prodromal symptoms like erythema marginatum (erythematous, serpentine,non-pruritic rash). An acute attack takes one day to peak and resolves in two to three days.
  • Acquired angioedema with C1 inhibitor deficiency – Acquired C1 inhibitor deficiency presents similarly to hereditary angioedema. However, the low C1 inhibitor in many cases is from an underlying lymphoproliferative disorder which increases protein consumption and an antibody against C1-INH causing overproduction of bradykinin.
  • The initial evaluation in the Emergency room – A specific drug and family history is needed along with screening blood work for C4 for hereditary angioedema and tryptase for angioedema with anaphylaxis. These labs when drawn during acute attacks are useful during follow-ups. In the case of anaphylaxis, (tryptase is normal in hereditary angioedema one and two but will be elevated in cases of anaphylaxis and other mast cell disorders associated with angioedema). Flexible fiberoptic laryngoscopy may be done to evaluate the involvement of the tongue and larynx in patients with head, neck and upper airway symptoms.Clear differentiation between histamine-induced vs. bradykinin-induced angioedema can be life-saving.
  • Hereditary angioedema type 1 – C1 inhibitor function low, C1-Inhibitor level low, and C4 level low
  • Hereditary angioedema type 2 – C1 inhibitor function and C4 level will be low, but the C1 inhibitor level will be normal or high.Confirm them by repeating the blood test.
  • Acquired C1-inhibitor deficiency – Low C1-inhibitor antigen and function and low C1q.

Treatment of Angioedema

Initially, the cause of angioedema is likely to be unknown, the mainstays of initial medical management include supplemental oxygen, a parenteral H-blocker, a parenteral steroid, and intramuscular epinephrine. Unfortunately, in AAE standard regimens of epinephrine, corticosteroids and antihistamines do not have much effect and are not recommended for AAE. Administration of plasma-derived or recombinant C1-INH can resolve acute attacks, but some patients will become non-responsive.

  • Icatibant, a bradykinin B2 receptor antagonist –  has been shown to provide symptoms relief in AAE and was well tolerated. Icatibant is an effective home-based, on-demand treatment. Before any high-risk procedure in high-risk patients, it is essential to give short-term prophylaxis. Anesthetists must be aware of the guidelines for HANE and AANE. C1 esterase inhibitor is the first-line long-term prophylaxis and androgens are used as second-line prophylaxis. The majority of AANE cases are asymptomatic and respond to immunochemotherapy.
  • Ecallantide, a potent bradykinin pathway inhibitor – has also shown promise in the treatment of AAE, providing another alternative in the treatment of patients with resistance to C1-INH replacement.
  • Fresh frozen plasma (FFP) – may be used. Although effective (at least in HAE), it at times will lack efficacy or even cause a sudden worsening of the patient’s symptoms. There is also the risk of viral transmission to consider.
  • Rituximab  – efficacy has been shown to be inconstant in AAE. It is also important to consider that treatment of an underlying causative condition can result in improvement of AAE.
  • Antihistamine, corticosteroids, and epinephrine – are treatments of histaminergic angioedema.Treatment of Bradykinin-mediated angioedema is often resistant to standard therapies such as epinephrine, glucocorticoids, or antihistamines.
  • Hereditary angioedema (On-demand treatment) – Treat airway via intubation or surgical airway intervention. Treatment should be as early as possible. Hereditary angioedema attacks should be treated with C1 Inhibitor concentrate,  ecallantide (Kallikrein inhibitor), or icatibant (bradykinin-receptor antagonist). Icatibant is an effective home-based, on-demand treatment.
  • Pre-procedural (short-term) prophylaxis – Short-term prophylaxis before high-risk procedures in high-risk individuals.  Anesthesiologists must be aware of guideline-based treatment for hereditary and acquired angioedema. 
  • Long-term prophylaxis – C1 Inhibitor is the first-line long-term prophylaxis, while the androgens are used as second-line agents.
  • Hereditary angioedema with mutations in the F12 gene is treated as follows – Discontinuing trigger factors (estrogen-containing oral contraceptives, hormonal replacement therapy, angiotensin-converting enzyme inhibitors). Treating with plasma-derived C1 inhibitor for acute attacks.  Preventing attacks with progestins, tranexamic acid, and danazol.
  • Acquired angioedema related to angiotensin-converting enzyme (ACE) inhibitor – Treatment is with antihistamines, epinephrine, and glucocorticoids. Care should be taken to stop the offending ACE inhibitor, and the patient should be not rechallenged with any of the ACE inhibitors in the future.
  • Acquired C1 inhibitor deficiency angioedema  – The majority of cases are asymptomatic and respond to immunochemotherapy Treatment of acute attacks with icatibant and plasma-derived C1 inhibitor concentrate, and prophylaxis is with rituximab with or without chemotherapy and splenectomy.
  • Antifibrinolytics and androgens – Two other therapies, which have recently fallen out of favor, include antifibrinolytics and androgens. Androgens induce the production of C1-inhibitor and are very effective in preventing attacks, but have no benefit in treating an attack. Short-term prophylaxis is tolerated well and is effective for pre-procedure of other short-term events; however, the toxicity of androgens limits their use for long-term prophylaxis. Most experts recommend not exceeding 200 mg of Danocrine a day. Adverse effects include hyperlipidemia, obesity, androgenic effects in females, and disposition problems.
  • Antifibrinolytics – include tranexamic acid and aminocaproic acid, and both have minimal effectiveness in the prevention and treatment of attacks. Presently, multiple other therapies are in the investigation including an oral kallikrein inhibitor and a monoclonal antibody that inhibits kallikrein. Table one includes the drugs that are currently available, and that may be available in the next few years.

Special considerations

  • In pregnancy, the recommended therapy is plasma-derived nano-filtered C1-inhibitor, however, in acute episodes, bradykinin receptor antagonist Icatibant can be used as it is safe with no maternal and fetal adverse effects.
  • In the pediatric population, the doses include 500 units (10-25 kg weighed patients), 1000 units, and 1500 Units in patients weighing more than 25kg.
  • Plasma-derived C1 esterase inhibitor is safe and efficacious in pediatric patients below 12 years.
  • Icatibant is a well-tolerated medication in the pediatric group and might have a role in treating angiotensin II receptor blocker-induced angioedema.
  • Recombinant human C1 esterase inhibitor for acute hereditary angioedema treatment has a persistent response for three days.
  • C1 inhibitor is used in the acute management of hereditary angioedema-associated pancreatitis.
  • Patients with life-threatening orolingual angioedema who are treated with recombinant tissue plasminogen activator infusion, have a rapid response after using icatibant treatment.
  • Concurrent use of angiotensin-converting enzyme inhibitors with, dipeptidyl peptidase-4 inhibitors should be monitored closely as dipeptidyl peptidase-4 is also a major enzyme in the degradation pathway of bradykinin like an angiotensin-converting enzyme.
  • Use of liquid steroids in patients with severe urticaria-associated angioedema in a setting of severe dysphagia secondary to anaphylaxis.
  • Use of omalizumab (Anti-immunoglobulin-E antibody) in Idiopathic non-histaminergic acquired angioedema, which is a rare disease resistant to antihistamines.

Hereditary angioedema

Acute episodes of hereditary angioedema do not respond to adrenaline, antihistamine and corticosteroids. Most acute episodes of Type I and II hereditary angioedema are not life-threatening.

  • The mainstay of emergency medical treatment is intravenous C1 inhibitor concentrate (a blood product).
  • If this is unavailable, fresh frozen plasma can be infused, but this occasionally exacerbates angioedema.
  • Icatibant, a synthetic peptidomimetic drug, and bradykinin B2 receptor antagonist can be used in emergencies for the symptomatic treatment of acute attacks of hereditary angioedema in adults with C1-esterase-inhibitor deficiency. It was approved by the FDA in 2011. In New Zealand, it is available for home use on Special Authority application.
  • Ecallantide is a potent and selective human plasma kallikrein inhibitor that is also indicated for the symptomatic treatment of hereditary angioedema, approved for use by the FDA in 2009. It is a protease that is responsible for liberating bradykinin from its precursor kininogen. Ecallantide has been reported to cause anaphylaxis in 4% of cases and thus has a black box warning in the USA.

The chance of an attack can be reduced with the following medications

  • C1 inhibitor concentrate infused an hour before a surgical procedure
  • Anabolic steroids (stanazolol, oxandrolone, and danazol) increase circulating levels of normal functional C1 inhibitor. These have ‘male-like hormonal activity, so may cause weight gain, menstrual irregularities, and virilism.
  • Tranexamic acid has been used in pre-pubertal children and may be effective in Type III hereditary angioedema.
  • A monoclonal antibody, lanadelumab, which inhibits active plasma kallikrein, has been approved in the USA for the prevention of hereditary angioedema attacks.

Complications

  • Critical airway occlusion resulting in death
  • Acute laryngeal, pharynx, and tongue swelling
  • Death from asphyxiation
  • Hereditary angioedema associated pancreatitis
  • Physicians should be mindful of cardiovascular instability including bradycardia after recombinant tissue plasminogen inhibitor in patients who take angiotensin-converting enzyme inhibitors

References

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Kraurosis Vulva – Causes, Symptoms, Diagnosis, Treatment

Kraurosis vulva is a cutaneous condition characterized by atrophy and shrinkage of the skin of the vagina and vulva often accompanied by a chronic inflammatory reaction in the deeper tissues.[rx]

Lichen Sclerosis (LS) is a chronic inflammatory disease. It was first described by Hallopeau in 1881.  In 1976, the International Society for the Study of Vulvovaginal Disease adopted the term lichen sclerosis. LS is a mucocutaneous autoimmune disorder characterized by hypopigmentation and skin atrophy. It involves most commonly the genital skin, less often the extragenital sites. LS is more common in women than in men. It may cause phimosis or scarring of the vaginal introitus. The diagnosis is based on the clinical features, but it is often confirmed by biopsy. The lesions can evolve towards the destruction of anatomic structures, functional impairment, and a potential risk for malignant evolution. Thus, treatment and long term follow-up are mandatory.

Lichen sclerosis et atrophicus (LSA) or, simply, lichen sclerosis, is a disorder characterized by an atrophic epidermis, altered collagen in the superficial dermis, and an overlying white or white-blue appearance to the skin. The terms balanitis xerotica obliterans and kraurosis vulvae have been applied to genital lesions of LSA in men and women, respectively. The pathogenesis of LSA is poorly understood, but the increased incidence of LSA with other autoimmune diseases (e.g., morphea) and the presence of autoantibodies directed against extracellular matrix protein 1 suggest LSA is an autoimmune disorder.

Another Name

Since then, multiple names have been used to describe this condition such as leukoplakia, kraurosis vulvae, balanitis xerotica obliterans, and lichen sclerosis atrophicus.

Causes of Lichen Sclerosis

  • LS is considered to be an autoimmune condition; however, its etiology remains unclear. Given its association with other autoimmune diseases such as alopecia areata, vitiligo, autoimmune thyroiditis, and pernicious anemia, its etiology is probably multifactorial. Further etiologic factors have been implicated such as genetic susceptibility, infectious agents such as spirochetes, sex hormone, and the Koebner phenomenon. Furthermore, recent data demonstrated a high prevalence of lichen sclerosis associated with morphea. 
  • The underlying pathogenesis of LS includes an infiltrate of activated T cells releasing interleukin 4 (IL 4) and transforming growth factor β (TGF β). So, these cytokines activate fibroblasts producing significantly altered collagen leading to fibrosis. Besides, the pathogenesis includes vascular damage by the decrease in the number of capillaries.
  • Interleukin 1 (IL 1) and interleukin one receptor antagonist (IL 1ra) may also be included in the pathogenesis of lichen sclerosis as well as an increased number of monoclonal T lymphocyte CD4 +, lymphocyte T dendritic CD1a + cells, macrophages, mast cells, and decreased number of T lymphocyte CD3 +.
  • Another hypothesis has been suggested such as an increased number of circulating IgG autoantibodies targeting extracellular matrix 1 (ECM 1) protein leading to widespread deposition of hyaline material in the dermis. 

Symptoms of Lichen Sclerosis

  • The reduction in structural complexity of vulvar tissue commonly occurs with advancing age, which is manifest externally by drying, shriveling and white patches. Formally known as kraurosis vulvae.
  • Often asymptomatic; less commonly, itching, dyspareunia, dysuria and tenderness.
  • LSA may present at any age, from pediatric to geriatric age groups.
  • The condition is more common in women.
  • The anogenital region alone is affected in about 50% of cases, the anogenital and extragenital disease is present in about 25% of cases, and only extragenital areas are affected in about 25% of cases.
  • Extragenital LSA is common on the neck, wrists, and inframammary areas.
  • Anogenital LSA in girls and women often demonstrates an hourglass-like or figure-eight configuration that surrounds the genitalia and anal region.
  • Early LSA may be pruritic or asymptomatic.
  • Primary lesions quickly evolve into white atrophic areas, with variable telangiectasias.
  • Some cases demonstrate the surrounding brown hyperpigmentation.
  • Variable features include hyperkeratosis and follicular plugging, fissures, blister formation hemorrhage, and ulceration.
  • Phimosis is a complication in uncircumcised men, and urethral stricture may also occur.
  • Squamous cell carcinoma is a rare complication with chronic genital LSA.

Diagnosis of Lichen Sclerosis

LS has a specific histologic pattern characterized by a band-like lymphocytic infiltrate below a zone of dermal edema and orthokeratotic hyperkeratosis. Histopathology findings vary depending on disease duration. In earlier stages, it shows vacuolar degeneration of the basal layer, hyalinization of subepithelial collagen, decreased elastic fibers in the upper dermis, and dilated blood vessels under the basement membrane. In older lesions, histology shows a reduced number of mononuclear cells and dispersed patchy islands of mononuclear cells within the hyalinized dermis.

History and Physical

LS affects most commonly the genital site and less often the extragenital area. Typical lesions begin as a sharply demarcated erythema that becomes thin, hypopigmented, ivory-white, porcelain-like, and sclerotic plaques. Plaques may become later thickened due to repeated excoriations. Itch is the main symptom and is often worse at night. Other lesions may include telangiectasias, purpura, fissures, ulcerations, and edema. Typical complaints commonly include significant pruritus, local burning sensation, pain, painful defecation. Constipation is frequent in children but rarely seen in adults. However, lesions can be asymptomatic. Genital lesions begin around the periclitoral hood. The affected area varies from a small and single area to a large area involving the entire region of the vulva, perineum, and perianus assuming a typical aspect of ‘keyhole sign.’

However, LS usually spares the vagina and cervix. In girls, LS presents commonly with irritation and soreness although it can mimic sexual abuse. However, LS and sexual abuse can coexist. Visual lichen sclerosis exhibits the Koebner phenomenon at sites of trauma; sexual abuse can aggravate lesions of LS.  Male genital LS (boys and men) occurs in the foreskin, glans penis, and the coronal sulcus penis. Extragenital lesions occur on any part of the skin and usually asymptomatic. The most commonly involved areas are inframammary areas, neck, wrists, thighs, upper back, and shoulders. The involvement of the oral mucosa appears clinically as bluish-white papules on the buccal mucosa or under the tongue.

Evaluation

The diagnosis is based on taking a careful history including the autoimmune diseases in the patient and family, the examination of the mucosas, extragenital skin, and completed by a gynecological exam. Thus, the diagnosis of LS is usually clinical. But in some cases, a biopsy can be performed. However, atypical histology does not rule out the diagnosis.

Biopsies should be performed in case of:

  • Atypical clinical presentation
  • Suspected malignancy
  • Nonresponse, after an appropriate duration, to recommended first-line treatment

The workup of the condition should include investigation of thyroid function, and according to symptoms investigation of the other autoimmune diseases.

Treatment of Lichen Sclerosis

The aims of the treatment are relief of the symptoms, stopping the atrophy, prevention of scar formation, and anatomical distortion, as well as malignant transformation. The therapy includes general care, topical treatments, systemic treatments, and surgical procedures. It is fundamental to inform the patient to avoid the use of irritating products such as soap and to prefer emollients to break the itch-stretch cycle.

For genital LS, the gold standard treatment is three months application of high potency topical steroids (clobetasol propionate). Second-line therapies include topical calcineurin inhibitors and imiquimod. In men, early circumcision may be recommended. Surgery is indicated only for the treatment of complications associated with lichen sclerosus. For extragenital LS, therapeutic modalities are limited and include phototherapy, ultrapotent topical steroids, tacrolimus ointment 0, 1%, and systemic steroids or methotrexate. Follow-up examinations must be kept indefinitely. 

There is no definitive cure for LS.[] Behavior change, such as good hygiene and minimizing scratching, is an important part of treatment,[] so a more realistic goal is to control pruritus rather than resolution of the lesion. Various treatment modalities have been tried with varied results.


Topical Treatment

Hormonal therapy

  • Estrogen is an effective treatment for postmenopausal vulvovaginal atrophy and should be considered in women with dyspareunia, labial fusion, or epidermal thinning due to estrogen deficiency but not as primary therapy of LS. Moisturizers and estrogens help in dryness and atrophy
  • Topical testosterone (2%) and progesterone (2%) were mainstays of treatment for decades and were reported to induce remission of LS. It acts by reducing inflammation and helps to relieve symptoms and in some cases, resolves the lesion, but androgenic side effects such as clitoral enlargement, hirsutism, acne vulgaris, and amenorrhea are common.

Topical steroids

VLS responds to ultrapotent topical corticosteroids, i.e, clobetasol propionate or mometasone furoate, though the clinical appearance does not reverse, the patient gets symptomatic relief and it prevents scarring. Clobetasol or clobetasol propionate 0.05% ointment is a Class I superpotent topical steroid which suppresses mitosis, increases the synthesis of proteins, decreases inflammation, and causes vasoconstriction. It is given daily at night for 6–12 weeks and then one to three times per week for maintenance.

Females should be evaluated regularly to see for skin atrophy or any malignant change. Prepubertal LS in girls may resolve spontaneously although some of them may suffer from various types of vulvodynia in adulthood.

Topical calcineurin inhibitors

Tacrolimus (0.1%) and pimecrolimus (1%) have a role as maintenance therapy but not as effective as potent topical corticosteroids and may be useful as alternative treatment options. It reduces itching and inflammation by suppressing the release of cytokines from T-cells and inhibits transcription for genes that encode interleukin (IL)-3, IL-4, IL-5, granulocyte-macrophage colony-stimulating factor, and tumor necrosis factor-alpha, which are involved in the early stages of T-cell activation. It also inhibits the release of preformed mediators from skin mast cells and basophils and downregulates the expression of FCeRI on Langerhans cells. Pimecrolimus is derived from ascomycin, a natural substance produced by Streptomyces hygroscopicus var. ascomycetous which selectively inhibits the production and release of inflammatory cytokines from activated T-cells by binding to cytosolic immunophilin receptor macrophilia-12. The resulting complex inhibits phosphatase calcineurin, thus blocking T-cell activation and cytokine release.[]

Topical retinoids

Topical tretinoin (0.025%) and tazarotene (0.01%) have off-label indications in LS. They act by downregulation of fibroblast function. Especially in genital areas, short-contact therapy is used in which the gel is initially applied for 15 min and washed off. Every 2–3 weeks, application time is increased by 15 min until therapeutic effects are noted.

In an observational series, topical use of tretinoin improved the symptoms, gross appearance, and histopathologic features of LS with minimal side effects.[]

Vitamin D analogs

Calcitriol, calcipotriene, and calcipotriene plus betamethasone helped some patients with localized sclerotic diseases. They can be irritating over genitals which require alternate-day therapy and should be used with caution in patients having compromised renal function.[]

Topical TRPM8

There is a single case report of the use of topical Sicilian in LS with relief in pruritus. Iselin is a TRPM8 receptor antagonist similar to menthol but with a higher affinity to the TRPM8 receptor.[,]

Topical avocado and soybean extracts

Borghi et al.[] suggest topical avocado and soybean extracts as alternative treatments for mild-to-moderate LS in patients wishing to avoid corticosteroids.

Results in their study provide evidence that the topical and dietary supplements exert anti-inflammatory, antifibrotic, emollient, and lenitive actions and are effective alternatives in the treatment of mild-to-moderate VLS.

Topical oxatomide

It helps relieve pruritus through its antihistamine effects, but the course of the disease is not affected.[]

Intralesional therapy

Injection of triamcinolone acetonide 20 mg directly into the thickened hypertrophic plaques of VLS once per month for 3 months[] after topical anesthetic help to minimize patient discomfort. Intralesional injection of adalimumab has also found to be beneficial.[]

Lights and Lasers

Phototherapy

Narrow-band ultraviolet B and psoralen plus ultraviolet A

A single study of ultraviolet A1 (UVA1) in seven women with VLS that had not been controlled by topical steroids[] reported initial improvement in five patients although two relapsed and the others required ongoing treatment with topical steroids. Studies have compared ultrapotent topical corticosteroids with the calcineurin inhibitors showing more efficacy of clobetasol propionate which works better compared with UVA1 phototherapy.

Photodynamic therapy using a photosensitizer

Successful treatment of VLS with photodynamic therapy (PDT) has been reported.[] In an open study of ten patients treated with two sessions of PDT, all patients reported some improvement in symptoms of VLS (itching, burning, and pain).[]

In an open study of PDT for VLS (topical 20% 5-aminolevulinic acid, argon laser light, and one to three treatments), 10 of 12 patients derived significant improvement.[] It caused significant burning although itching improved in 8 of 12 women. Another study demonstrated good symptomatic benefit in six of ten patients treated with aminolevulinic acid-PDT using a bioadhesive patch.[]

Cryotherapy

Cryotherapy of affected genital lesions after one or a series of treatments shows improvement. In one small study of 12 patients with VLS and severe intractable itch, 75% obtained symptom relief with cryotherapy.[]

Laser

Tissue-vaporizing carbon dioxide lasers, nonablative lasers such as the pulsed dye, and erbium-doped yttrium aluminum garnet lasers have been reported to benefit LS symptomatically but did not stop the disease from recurring. There is a report showing benefit in 17 out of 31 cases of untreated LS using frequencies of 5–8 MHz with focused ultrasound.[]

Systemic Therapy

Retinoids

Retinoids appear to reduce connective tissue degeneration in LS. However, the use of these agents is limited by significant and potentially harmful side effects including cheilitis, xerosis, teratogenicity, elevated liver enzymes, hypertriglyceridemia, abdominal pain, and alopecia. Isotretinoin decreases sebaceous gland size and sebum production. It may inhibit sebaceous gland differentiation and abnormal keratinization.

Oral acitretin (20–30 mg/day for 16 weeks) was effective in one randomized trial.[] Doses of 8–30 mg daily for 4 months have been used which gave benefits both in symptoms and also induce resolution of lesions. The mechanism of action of systemic retinoids in LS particularly in genital LS is not clear; mostly, it acts by downregulation of fibroblast function.

Hydroxychloroquine

It is reported to be effective in widespread genital and extragenital LSA, with conflicting results.[]

Hydroxycarbamide (hydroxyurea)

Hydroxycarbamide is an antineoplastic drug used in myeloproliferative disorders. It inhibits T-lymphocyte proliferation and gamma interferon production and has antiretroviral properties. It is used in LS in the dose of 1 g daily reducing pruritus and soreness in 6 months.[]

Cyclotron

The cyclotron is a low molecular weight interferon-inducing substance that has antiviral, immunomodulating, and anti-inflammatory effects. A prospective randomized study involved sixty patients with chronic dystrophic diseases of the vulva (45–65 years); cyclotron given intramuscular on days 1, 2, 4, 6, 8, 10, 12, 16, 20, and 23 was reported to induce rapid remission, improvement of QoL, and psychosocial function.[]

Cyclosporine

Cyclosporine has been used in only ten patients with VLS.[] Three patients had a significant improvement, five had some response, and two had no response.

Potassium Para-aminobenzoate

One report of five patients with LS at various sites and resistant to numerous other therapies documented good improvement with potassium para-aminobenzoate (4–24 g daily, in divided doses) in all five.[]

A small number of women initially have a partial response to medical treatment but have ongoing burning, irritation, and pain. In these cases, obtain cultures to exclude superinfection by Staphylococcus, Streptococcus, or Candida. The patient may have bacterial cellulitis, vulvar candidiasis, or vaginal candidiasis, which requires treatment with appropriate antibiotics or antimycotic drugs.


Diet

Twenty-three patients also received dietary supplements with Vitamin E and para-aminobenzoic acid showed improvement.[]

Surgery

VLS can be surgically excised, but mutilating gynecologic surgery is usually not recommended. The rationale behind surgical therapy is primarily to treat those patients who did not respond or responded poorly to medical treatment, secondarily to postinflammatory sequelae, and prevent the development of invasive carcinoma of the vulva.

Surgical intervention in LS is done to release a buried clitoris, to separate fused labia, or to widen a narrowed introitus in the case of pain or sexual dysfunction. V-Y advancement flap is an effective method for the reconstruction of the perineal region. This technique will allow the expansion of the vaginal orifice with good cosmetic results and rapid healing after surgery.

Introit stenosis, posterior fissuring, and scarring at the fourchette can be treated by vulvoperineoplasty.[] Since vaginal tissue is not affected by LS, part of the vaginal wall is used in the repair to prevent recurrent adhesions and fissuring at the introitus. Adhesions at the fourchette which cause dyspareunia can be extirpated. Adhesions burying the clitoris can result in the formation of painful pseudocysts. Clitoral adhesions are released with delicate knife strokes. Reformation of adhesions can be prevented by resection of a fragment of the clitoral hood in the shape of a tricorn.[]

Other points that need to be considered treating VLS are as follows:

  • Menopausal women may have symptoms related to atrophy and dryness, which will respond to topical estrogen cream and moisturizers
  • A diagnosis of superimposed vulvodynia should be considered if pain persists despite resolution of pruritus and dermal changes. It is likely that vulvodynia represents neuropathic pain, which is pain arising from abnormal neural activity secondary to disease, irritation, or injury of the nervous system that persists in the absence of ongoing disease or acute injury.
  • An allergy to the topical medication may be present. A topical steroid with a different base or consultation with an allergy specialist should be considered.

Counseling

According to the National Vulvodynia Association, women with VLS or any vulvovaginal condition experience feelings of isolation, hopelessness, depression, anxiety, anger, and low self-image. Some women are unable to continue working, any physical activity, or sexual relations.

Psychological counseling is very much needed in patients of VLS. Education relating to sexual dysfunction and dyspareunia may be required. Patients should be educated on what changes (e.g., ulceration) might indicate malignant transformation so that immediate consultation can be done.

Counseling involves self-care to be taken by patient themselves such as:

  • Avoid washing with soap or to use an emollient soap
  • Carefully dry the area after passing urine to reduce the contact of urine or using a moisturizer or soft paraffin as a barrier cream to protect skin from exposure to urine
  • If sexual intercourse is painful because of tightening at the entrance to the vagina, use of lubricants and vaginal dilators if required
  • Keep an eye on your skin. Regular self-examination is very important. If any skin change develops which does not respond to steroid creams, or there is any skin thickening, soreness, or ulceration lasting more than 2 weeks, consult without delay and get a biopsy done to rule out skin cancer.

References

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[Total: 0 Average: 0]

Lichen Sclerosis Atrophicus – Causes, Symptoms, Treatment

Lichen Sclerosis Atrophicus (LSA) or, simply, lichen sclerosis, is a disorder characterized by an atrophic epidermis, altered collagen in the superficial dermis, and an overlying white or white-blue appearance to the skin. The terms balanitis xerotica obliterans and kraurosis vulvae have been applied to genital lesions of LSA in men and women, respectively. The pathogenesis of LSA is poorly understood, but the increased incidence of LSA with other autoimmune diseases (e.g., morphea) and the presence of autoantibodies directed against extracellular matrix protein 1 suggest LSA is an autoimmune disorder.

Kraurosis vulva is a cutaneous condition characterized by atrophy and shrinkage of the skin of the vagina and vulva often accompanied by a chronic inflammatory reaction in the deeper tissues.[rx]

Lichen Sclerosis (LS) is a chronic inflammatory disease. It was first described by Hallopeau in 1881.  In 1976, the International Society for the Study of Vulvovaginal Disease adopted the term lichen sclerosis. LS is a mucocutaneous autoimmune disorder characterized by hypopigmentation and skin atrophy. It involves most commonly the genital skin, less often the extragenital sites. LS is more common in women than in men. It may cause phimosis or scarring of the vaginal introitus. The diagnosis is based on the clinical features, but it is often confirmed by biopsy. The lesions can evolve towards the destruction of anatomic structures, functional impairment, and a potential risk for malignant evolution. Thus, treatment and long term follow-up are mandatory.

Another Name

Since then, multiple names have been used to describe this condition such as leukoplakia, kraurosis vulvae, balanitis xerotica obliterans, and lichen sclerosis atrophicus.

Causes of Lichen Sclerosis Atrophicus

  • LS is considered to be an autoimmune condition; however, its etiology remains unclear. Given its association with other autoimmune diseases such as alopecia areata, vitiligo, autoimmune thyroiditis, and pernicious anemia, its etiology is probably multifactorial. Further etiologic factors have been implicated such as genetic susceptibility, infectious agents such as spirochetes, sex hormone, and the Koebner phenomenon. Furthermore, recent data demonstrated a high prevalence of lichen sclerosis associated with morphea. 
  • The underlying pathogenesis of LS includes an infiltrate of activated T cells releasing interleukin 4 (IL 4) and transforming growth factor β (TGF β). So, these cytokines activate fibroblasts producing significantly altered collagen leading to fibrosis. Besides, the pathogenesis includes vascular damage by the decrease in the number of capillaries.
  • Interleukin 1 (IL 1) and interleukin one receptor antagonist (IL 1ra) may also be included in the pathogenesis of lichen sclerosis as well as an increased number of monoclonal T lymphocyte CD4 +, lymphocyte T dendritic CD1a + cells, macrophages, mast cells, and decreased number of T lymphocyte CD3 +.
  • Another hypothesis has been suggested such as an increased number of circulating IgG autoantibodies targeting extracellular matrix 1 (ECM 1) protein leading to widespread deposition of hyaline material in the dermis. 

Symptoms of Lichen Sclerosis Atrophicus

  • The reduction in structural complexity of vulvar tissue commonly occurs with advancing age, which is manifest externally by drying, shriveling and white patches. Formally known as kraurosis vulvae.
  • Often asymptomatic; less commonly, itching, dyspareunia, dysuria and tenderness.
  • LSA may present at any age, from pediatric to geriatric age groups.
  • The condition is more common in women.
  • The anogenital region alone is affected in about 50% of cases, the anogenital and extragenital disease is present in about 25% of cases, and only extragenital areas are affected in about 25% of cases.
  • Extragenital LSA is common on the neck, wrists, and inframammary areas.
  • Anogenital LSA in girls and women often demonstrates an hourglass-like or figure-eight configuration that surrounds the genitalia and anal region.
  • Early LSA may be pruritic or asymptomatic.
  • Primary lesions quickly evolve into white atrophic areas, with variable telangiectasias.
  • Some cases demonstrate the surrounding brown hyperpigmentation.
  • Variable features include hyperkeratosis and follicular plugging, fissures, blister formation hemorrhage, and ulceration.
  • Phimosis is a complication in uncircumcised men, and urethral stricture may also occur.
  • Squamous cell carcinoma is a rare complication with chronic genital LSA.

Diagnosis of Lichen Sclerosis Atrophicus

LS has a specific histologic pattern characterized by a band-like lymphocytic infiltrate below a zone of dermal edema and orthokeratotic hyperkeratosis. Histopathology findings vary depending on disease duration. In earlier stages, it shows vacuolar degeneration of the basal layer, hyalinization of subepithelial collagen, decreased elastic fibers in the upper dermis, and dilated blood vessels under the basement membrane. In older lesions, histology shows a reduced number of mononuclear cells and dispersed patchy islands of mononuclear cells within the hyalinized dermis.

History and Physical

LS affects most commonly the genital site and less often the extragenital area. Typical lesions begin as a sharply demarcated erythema that becomes thin, hypopigmented, ivory-white, porcelain-like, and sclerotic plaques. Plaques may become later thickened due to repeated excoriations. Itch is the main symptom and is often worse at night. Other lesions may include telangiectasias, purpura, fissures, ulcerations, and edema. Typical complaints commonly include significant pruritus, local burning sensation, pain, painful defecation. Constipation is frequent in children but rarely seen in adults. However, lesions can be asymptomatic. Genital lesions begin around the periclitoral hood. The affected area varies from a small and single area to a large area involving the entire region of the vulva, perineum, and perianus assuming a typical aspect of ‘keyhole sign.’

However, LS usually spares the vagina and cervix. In girls, LS presents commonly with irritation and soreness although it can mimic sexual abuse. However, LS and sexual abuse can coexist. Visual lichen sclerosis exhibits the Koebner phenomenon at sites of trauma; sexual abuse can aggravate lesions of LS.  Male genital LS (boys and men) occurs in the foreskin, glans penis, and the coronal sulcus penis. Extragenital lesions occur on any part of the skin and usually asymptomatic. The most commonly involved areas are inframammary areas, neck, wrists, thighs, upper back, and shoulders. The involvement of the oral mucosa appears clinically as bluish-white papules on the buccal mucosa or under the tongue.

Evaluation

The diagnosis is based on taking a careful history including the autoimmune diseases in the patient and family, the examination of the mucosas, extragenital skin, and completed by a gynecological exam. Thus, the diagnosis of LS is usually clinical. But in some cases, a biopsy can be performed. However, atypical histology does not rule out the diagnosis.

Biopsies should be performed in case of:

  • Atypical clinical presentation
  • Suspected malignancy
  • Nonresponse, after an appropriate duration, to recommended first-line treatment

The workup of the condition should include investigation of thyroid function, and according to symptoms investigation of the other autoimmune diseases.

Treatment of Lichen Sclerosis Atrophicus

The aims of the treatment are relief of the symptoms, stopping the atrophy, prevention of scar formation, and anatomical distortion, as well as malignant transformation. The therapy includes general care, topical treatments, systemic treatments, and surgical procedures. It is fundamental to inform the patient to avoid the use of irritating products such as soap and to prefer emollients to break the itch-stretch cycle.

For genital LS, the gold standard treatment is three months application of high potency topical steroids (clobetasol propionate). Second-line therapies include topical calcineurin inhibitors and imiquimod. In men, early circumcision may be recommended. Surgery is indicated only for the treatment of complications associated with lichen sclerosus. For extragenital LS, therapeutic modalities are limited and include phototherapy, ultrapotent topical steroids, tacrolimus ointment 0, 1%, and systemic steroids or methotrexate. Follow-up examinations must be kept indefinitely. 

There is no definitive cure for LS.[] Behavior change, such as good hygiene and minimizing scratching, is an important part of treatment,[] so a more realistic goal is to control pruritus rather than resolution of the lesion. Various treatment modalities have been tried with varied results.


Topical Treatment

Hormonal therapy

  • Estrogen is an effective treatment for postmenopausal vulvovaginal atrophy and should be considered in women with dyspareunia, labial fusion, or epidermal thinning due to estrogen deficiency but not as primary therapy of LS. Moisturizers and estrogens help in dryness and atrophy
  • Topical testosterone (2%) and progesterone (2%) were mainstays of treatment for decades and were reported to induce remission of LS. It acts by reducing inflammation and helps to relieve symptoms and in some cases, resolves the lesion, but androgenic side effects such as clitoral enlargement, hirsutism, acne vulgaris, and amenorrhea are common.

Topical steroids

VLS responds to ultrapotent topical corticosteroids, i.e, clobetasol propionate or mometasone furoate, though the clinical appearance does not reverse, the patient gets symptomatic relief and it prevents scarring. Clobetasol or clobetasol propionate 0.05% ointment is a Class I superpotent topical steroid which suppresses mitosis, increases the synthesis of proteins, decreases inflammation, and causes vasoconstriction. It is given daily at night for 6–12 weeks and then one to three times per week for maintenance.

Females should be evaluated regularly to see for skin atrophy or any malignant change. Prepubertal LS in girls may resolve spontaneously although some of them may suffer from various types of vulvodynia in adulthood.

Topical calcineurin inhibitors

Tacrolimus (0.1%) and pimecrolimus (1%) have a role as maintenance therapy but not as effective as potent topical corticosteroids and may be useful as alternative treatment options. It reduces itching and inflammation by suppressing the release of cytokines from T-cells and inhibits transcription for genes that encode interleukin (IL)-3, IL-4, IL-5, granulocyte-macrophage colony-stimulating factor, and tumor necrosis factor-alpha, which are involved in the early stages of T-cell activation. It also inhibits the release of preformed mediators from skin mast cells and basophils and downregulates the expression of FCeRI on Langerhans cells. Pimecrolimus is derived from ascomycin, a natural substance produced by Streptomyces hygroscopicus var. ascomycetous which selectively inhibits the production and release of inflammatory cytokines from activated T-cells by binding to cytosolic immunophilin receptor macrophilia-12. The resulting complex inhibits phosphatase calcineurin, thus blocking T-cell activation and cytokine release.[]

Topical retinoids

Topical tretinoin (0.025%) and tazarotene (0.01%) have off-label indications in LS. They act by downregulation of fibroblast function. Especially in genital areas, short-contact therapy is used in which the gel is initially applied for 15 min and washed off. Every 2–3 weeks, application time is increased by 15 min until therapeutic effects are noted.

In an observational series, topical use of tretinoin improved the symptoms, gross appearance, and histopathologic features of LS with minimal side effects.[]

Vitamin D analogs

Calcitriol, calcipotriene, and calcipotriene plus betamethasone helped some patients with localized sclerotic diseases. They can be irritating over genitals which require alternate-day therapy and should be used with caution in patients having compromised renal function.[]

Topical TRPM8

There is a single case report of the use of topical Sicilian in LS with relief in pruritus. Iselin is a TRPM8 receptor antagonist similar to menthol but with a higher affinity to the TRPM8 receptor.[,]

Topical avocado and soybean extracts

Borghi et al.[] suggest topical avocado and soybean extracts as alternative treatments for mild-to-moderate LS in patients wishing to avoid corticosteroids.

Results in their study provide evidence that the topical and dietary supplements exert anti-inflammatory, antifibrotic, emollient, and lenitive actions and are effective alternatives in the treatment of mild-to-moderate VLS.

Topical oxatomide

It helps relieve pruritus through its antihistamine effects, but the course of the disease is not affected.[]

Intralesional therapy

Injection of triamcinolone acetonide 20 mg directly into the thickened hypertrophic plaques of VLS once per month for 3 months[] after topical anesthetic help to minimize patient discomfort. Intralesional injection of adalimumab has also found to be beneficial.[]

Lights and Lasers

Phototherapy

Narrow-band ultraviolet B and psoralen plus ultraviolet A

A single study of ultraviolet A1 (UVA1) in seven women with VLS that had not been controlled by topical steroids[] reported initial improvement in five patients although two relapsed and the others required ongoing treatment with topical steroids. Studies have compared ultrapotent topical corticosteroids with the calcineurin inhibitors showing more efficacy of clobetasol propionate which works better compared with UVA1 phototherapy.

Photodynamic therapy using a photosensitizer

Successful treatment of VLS with photodynamic therapy (PDT) has been reported.[] In an open study of ten patients treated with two sessions of PDT, all patients reported some improvement in symptoms of VLS (itching, burning, and pain).[]

In an open study of PDT for VLS (topical 20% 5-aminolevulinic acid, argon laser light, and one to three treatments), 10 of 12 patients derived significant improvement.[] It caused significant burning although itching improved in 8 of 12 women. Another study demonstrated good symptomatic benefit in six of ten patients treated with aminolevulinic acid-PDT using a bioadhesive patch.[]

Cryotherapy

Cryotherapy of affected genital lesions after one or a series of treatments shows improvement. In one small study of 12 patients with VLS and severe intractable itch, 75% obtained symptom relief with cryotherapy.[]

Laser

Tissue-vaporizing carbon dioxide lasers, nonablative lasers such as the pulsed dye, and erbium-doped yttrium aluminum garnet lasers have been reported to benefit LS symptomatically but did not stop the disease from recurring. There is a report showing benefit in 17 out of 31 cases of untreated LS using frequencies of 5–8 MHz with focused ultrasound.[]

Systemic Therapy

Retinoids

Retinoids appear to reduce connective tissue degeneration in LS. However, the use of these agents is limited by significant and potentially harmful side effects including cheilitis, xerosis, teratogenicity, elevated liver enzymes, hypertriglyceridemia, abdominal pain, and alopecia. Isotretinoin decreases sebaceous gland size and sebum production. It may inhibit sebaceous gland differentiation and abnormal keratinization.

Oral acitretin (20–30 mg/day for 16 weeks) was effective in one randomized trial.[] Doses of 8–30 mg daily for 4 months have been used which gave benefits both in symptoms and also induce resolution of lesions. The mechanism of action of systemic retinoids in LS particularly in genital LS is not clear; mostly, it acts by downregulation of fibroblast function.

Hydroxychloroquine

It is reported to be effective in widespread genital and extragenital LSA, with conflicting results.[]

Hydroxycarbamide (hydroxyurea)

Hydroxycarbamide is an antineoplastic drug used in myeloproliferative disorders. It inhibits T-lymphocyte proliferation and gamma interferon production and has antiretroviral properties. It is used in LS in the dose of 1 g daily reducing pruritus and soreness in 6 months.[]

Cyclotron

The cyclotron is a low molecular weight interferon-inducing substance that has antiviral, immunomodulating, and anti-inflammatory effects. A prospective randomized study involved sixty patients with chronic dystrophic diseases of the vulva (45–65 years); cyclotron given intramuscular on days 1, 2, 4, 6, 8, 10, 12, 16, 20, and 23 was reported to induce rapid remission, improvement of QoL, and psychosocial function.[]

Cyclosporine

Cyclosporine has been used in only ten patients with VLS.[] Three patients had a significant improvement, five had some response, and two had no response.

Potassium Para-aminobenzoate

One report of five patients with LS at various sites and resistant to numerous other therapies documented good improvement with potassium para-aminobenzoate (4–24 g daily, in divided doses) in all five.[]

A small number of women initially have a partial response to medical treatment but have ongoing burning, irritation, and pain. In these cases, obtain cultures to exclude superinfection by Staphylococcus, Streptococcus, or Candida. The patient may have bacterial cellulitis, vulvar candidiasis, or vaginal candidiasis, which requires treatment with appropriate antibiotics or antimycotic drugs.


Diet

Twenty-three patients also received dietary supplements with Vitamin E and para-aminobenzoic acid showed improvement.[]

Surgery

VLS can be surgically excised, but mutilating gynecologic surgery is usually not recommended. The rationale behind surgical therapy is primarily to treat those patients who did not respond or responded poorly to medical treatment, secondarily to postinflammatory sequelae, and prevent the development of invasive carcinoma of the vulva.

Surgical intervention in LS is done to release a buried clitoris, to separate fused labia, or to widen a narrowed introitus in the case of pain or sexual dysfunction. V-Y advancement flap is an effective method for the reconstruction of the perineal region. This technique will allow the expansion of the vaginal orifice with good cosmetic results and rapid healing after surgery.

Introit stenosis, posterior fissuring, and scarring at the fourchette can be treated by vulvoperineoplasty.[] Since vaginal tissue is not affected by LS, part of the vaginal wall is used in the repair to prevent recurrent adhesions and fissuring at the introitus. Adhesions at the fourchette which cause dyspareunia can be extirpated. Adhesions burying the clitoris can result in the formation of painful pseudocysts. Clitoral adhesions are released with delicate knife strokes. Reformation of adhesions can be prevented by resection of a fragment of the clitoral hood in the shape of a tricorn.[]

Other points that need to be considered treating VLS are as follows:

  • Menopausal women may have symptoms related to atrophy and dryness, which will respond to topical estrogen cream and moisturizers
  • A diagnosis of superimposed vulvodynia should be considered if pain persists despite resolution of pruritus and dermal changes. It is likely that vulvodynia represents neuropathic pain, which is pain arising from abnormal neural activity secondary to disease, irritation, or injury of the nervous system that persists in the absence of ongoing disease or acute injury.
  • An allergy to the topical medication may be present. A topical steroid with a different base or consultation with an allergy specialist should be considered.

Counseling

According to the National Vulvodynia Association, women with VLS or any vulvovaginal condition experience feelings of isolation, hopelessness, depression, anxiety, anger, and low self-image. Some women are unable to continue working, any physical activity, or sexual relations.

Psychological counseling is very much needed in patients of VLS. Education relating to sexual dysfunction and dyspareunia may be required. Patients should be educated on what changes (e.g., ulceration) might indicate malignant transformation so that immediate consultation can be done.

Counseling involves self-care to be taken by patient themselves such as:

  • Avoid washing with soap or to use an emollient soap
  • Carefully dry the area after passing urine to reduce the contact of urine or using a moisturizer or soft paraffin as a barrier cream to protect skin from exposure to urine
  • If sexual intercourse is painful because of tightening at the entrance to the vagina, use of lubricants and vaginal dilators if required
  • Keep an eye on your skin. Regular self-examination is very important. If any skin change develops which does not respond to steroid creams, or there is any skin thickening, soreness, or ulceration lasting more than 2 weeks, consult without delay and get a biopsy done to rule out skin cancer.

References

If the article is helpful, please Click to Star Icon and Rate This Post!
[Total: 0 Average: 0]

Leukoplakia – Causes, Symptoms, Treatment

Leukoplakia/Lichen Sclerosis atrophicus (LSA) or, simply, lichen sclerosis, is a disorder characterized by an atrophic epidermis, altered collagen in the superficial dermis, and an overlying white or white-blue appearance to the skin. The terms balanitis xerotica obliterans and kraurosis vulvae have been applied to genital lesions of LSA in men and women, respectively. The pathogenesis of LSA is poorly understood, but the increased incidence of LSA with other autoimmune diseases (e.g., morphea) and the presence of autoantibodies directed against extracellular matrix protein 1 suggest LSA is an autoimmune disorder.

Kraurosis vulva is a cutaneous condition characterized by atrophy and shrinkage of the skin of the vagina and vulva often accompanied by a chronic inflammatory reaction in the deeper tissues.[rx]

Lichen Sclerosis (LS) is a chronic inflammatory disease. It was first described by Hallopeau in 1881.  In 1976, the International Society for the Study of Vulvovaginal Disease adopted the term lichen sclerosis. LS is a mucocutaneous autoimmune disorder characterized by hypopigmentation and skin atrophy. It involves most commonly the genital skin, less often the extragenital sites. LS is more common in women than in men. It may cause phimosis or scarring of the vaginal introitus. The diagnosis is based on the clinical features, but it is often confirmed by biopsy. The lesions can evolve towards the destruction of anatomic structures, functional impairment, and a potential risk for malignant evolution. Thus, treatment and long term follow-up are mandatory.

Another Name

Since then, multiple names have been used to describe this condition such as leukoplakia, kraurosis vulvae, balanitis xerotica obliterans, and lichen sclerosis atrophicus.

Causes of Leukoplakia

  • LS is considered to be an autoimmune condition; however, its etiology remains unclear. Given its association with other autoimmune diseases such as alopecia areata, vitiligo, autoimmune thyroiditis, and pernicious anemia, its etiology is probably multifactorial. Further etiologic factors have been implicated such as genetic susceptibility, infectious agents such as spirochetes, sex hormone, and the Koebner phenomenon. Furthermore, recent data demonstrated a high prevalence of lichen sclerosis associated with morphea. 
  • The underlying pathogenesis of LS includes an infiltrate of activated T cells releasing interleukin 4 (IL 4) and transforming growth factor β (TGF β). So, these cytokines activate fibroblasts producing significantly altered collagen leading to fibrosis. Besides, the pathogenesis includes vascular damage by the decrease in the number of capillaries.
  • Interleukin 1 (IL 1) and interleukin one receptor antagonist (IL 1ra) may also be included in the pathogenesis of lichen sclerosis as well as an increased number of monoclonal T lymphocyte CD4 +, lymphocyte T dendritic CD1a + cells, macrophages, mast cells, and decreased number of T lymphocyte CD3 +.
  • Another hypothesis has been suggested such as an increased number of circulating IgG autoantibodies targeting extracellular matrix 1 (ECM 1) protein leading to widespread deposition of hyaline material in the dermis. 

Symptoms of Leukoplakia

  • The reduction in structural complexity of vulvar tissue commonly occurs with advancing age, which is manifest externally by drying, shriveling and white patches. Formally known as kraurosis vulvae.
  • Often asymptomatic; less commonly, itching, dyspareunia, dysuria and tenderness.
  • LSA may present at any age, from pediatric to geriatric age groups.
  • The condition is more common in women.
  • The anogenital region alone is affected in about 50% of cases, the anogenital and extragenital disease is present in about 25% of cases, and only extragenital areas are affected in about 25% of cases.
  • Extragenital LSA is common on the neck, wrists, and inframammary areas.
  • Anogenital LSA in girls and women often demonstrates an hourglass-like or figure-eight configuration that surrounds the genitalia and anal region.
  • Early LSA may be pruritic or asymptomatic.
  • Primary lesions quickly evolve into white atrophic areas, with variable telangiectasias.
  • Some cases demonstrate the surrounding brown hyperpigmentation.
  • Variable features include hyperkeratosis and follicular plugging, fissures, blister formation hemorrhage, and ulceration.
  • Phimosis is a complication in uncircumcised men, and urethral stricture may also occur.
  • Squamous cell carcinoma is a rare complication with chronic genital LSA.

Diagnosis of Leukoplakia

LS has a specific histologic pattern characterized by a band-like lymphocytic infiltrate below a zone of dermal edema and orthokeratotic hyperkeratosis. Histopathology findings vary depending on disease duration. In earlier stages, it shows vacuolar degeneration of the basal layer, hyalinization of subepithelial collagen, decreased elastic fibers in the upper dermis, and dilated blood vessels under the basement membrane. In older lesions, histology shows a reduced number of mononuclear cells and dispersed patchy islands of mononuclear cells within the hyalinized dermis.

History and Physical

LS affects most commonly the genital site and less often the extragenital area. Typical lesions begin as a sharply demarcated erythema that becomes thin, hypopigmented, ivory-white, porcelain-like, and sclerotic plaques. Plaques may become later thickened due to repeated excoriations. Itch is the main symptom and is often worse at night. Other lesions may include telangiectasias, purpura, fissures, ulcerations, and edema. Typical complaints commonly include significant pruritus, local burning sensation, pain, painful defecation. Constipation is frequent in children but rarely seen in adults. However, lesions can be asymptomatic. Genital lesions begin around the periclitoral hood. The affected area varies from a small and single area to a large area involving the entire region of the vulva, perineum, and perianus assuming a typical aspect of ‘keyhole sign.’

However, LS usually spares the vagina and cervix. In girls, LS presents commonly with irritation and soreness although it can mimic sexual abuse. However, LS and sexual abuse can coexist. Visual lichen sclerosis exhibits the Koebner phenomenon at sites of trauma; sexual abuse can aggravate lesions of LS.  Male genital LS (boys and men) occurs in the foreskin, glans penis, and the coronal sulcus penis. Extragenital lesions occur on any part of the skin and usually asymptomatic. The most commonly involved areas are inframammary areas, neck, wrists, thighs, upper back, and shoulders. The involvement of the oral mucosa appears clinically as bluish-white papules on the buccal mucosa or under the tongue.

Evaluation

The diagnosis is based on taking a careful history including the autoimmune diseases in the patient and family, the examination of the mucosas, extragenital skin, and completed by a gynecological exam. Thus, the diagnosis of LS is usually clinical. But in some cases, a biopsy can be performed. However, atypical histology does not rule out the diagnosis.

Biopsies should be performed in case of:

  • Atypical clinical presentation
  • Suspected malignancy
  • Nonresponse, after an appropriate duration, to recommended first-line treatment

The workup of the condition should include investigation of thyroid function, and according to symptoms investigation of the other autoimmune diseases.

Treatment of Leukoplakia

The aims of the treatment are relief of the symptoms, stopping the atrophy, prevention of scar formation, and anatomical distortion, as well as malignant transformation. The therapy includes general care, topical treatments, systemic treatments, and surgical procedures. It is fundamental to inform the patient to avoid the use of irritating products such as soap and to prefer emollients to break the itch-stretch cycle.

For genital LS, the gold standard treatment is three months application of high potency topical steroids (clobetasol propionate). Second-line therapies include topical calcineurin inhibitors and imiquimod. In men, early circumcision may be recommended. Surgery is indicated only for the treatment of complications associated with lichen sclerosus. For extragenital LS, therapeutic modalities are limited and include phototherapy, ultrapotent topical steroids, tacrolimus ointment 0, 1%, and systemic steroids or methotrexate. Follow-up examinations must be kept indefinitely. 

There is no definitive cure for LS.[] Behavior change, such as good hygiene and minimizing scratching, is an important part of treatment,[] so a more realistic goal is to control pruritus rather than resolution of the lesion. Various treatment modalities have been tried with varied results.


Topical Treatment

Hormonal therapy

  • Estrogen is an effective treatment for postmenopausal vulvovaginal atrophy and should be considered in women with dyspareunia, labial fusion, or epidermal thinning due to estrogen deficiency but not as primary therapy of LS. Moisturizers and estrogens help in dryness and atrophy
  • Topical testosterone (2%) and progesterone (2%) were mainstays of treatment for decades and were reported to induce remission of LS. It acts by reducing inflammation and helps to relieve symptoms and in some cases, resolves the lesion, but androgenic side effects such as clitoral enlargement, hirsutism, acne vulgaris, and amenorrhea are common.

Topical steroids

VLS responds to ultrapotent topical corticosteroids, i.e, clobetasol propionate or mometasone furoate, though the clinical appearance does not reverse, the patient gets symptomatic relief and it prevents scarring. Clobetasol or clobetasol propionate 0.05% ointment is a Class I superpotent topical steroid which suppresses mitosis, increases the synthesis of proteins, decreases inflammation, and causes vasoconstriction. It is given daily at night for 6–12 weeks and then one to three times per week for maintenance.

Females should be evaluated regularly to see for skin atrophy or any malignant change. Prepubertal LS in girls may resolve spontaneously although some of them may suffer from various types of vulvodynia in adulthood.

Topical calcineurin inhibitors

Tacrolimus (0.1%) and pimecrolimus (1%) have a role as maintenance therapy but not as effective as potent topical corticosteroids and may be useful as alternative treatment options. It reduces itching and inflammation by suppressing the release of cytokines from T-cells and inhibits transcription for genes that encode interleukin (IL)-3, IL-4, IL-5, granulocyte-macrophage colony-stimulating factor, and tumor necrosis factor-alpha, which are involved in the early stages of T-cell activation. It also inhibits the release of preformed mediators from skin mast cells and basophils and downregulates the expression of FCeRI on Langerhans cells. Pimecrolimus is derived from ascomycin, a natural substance produced by Streptomyces hygroscopicus var. ascomycetous which selectively inhibits the production and release of inflammatory cytokines from activated T-cells by binding to cytosolic immunophilin receptor macrophilia-12. The resulting complex inhibits phosphatase calcineurin, thus blocking T-cell activation and cytokine release.[]

Topical retinoids

Topical tretinoin (0.025%) and tazarotene (0.01%) have off-label indications in LS. They act by downregulation of fibroblast function. Especially in genital areas, short-contact therapy is used in which the gel is initially applied for 15 min and washed off. Every 2–3 weeks, application time is increased by 15 min until therapeutic effects are noted.

In an observational series, topical use of tretinoin improved the symptoms, gross appearance, and histopathologic features of LS with minimal side effects.[]

Vitamin D analogs

Calcitriol, calcipotriene, and calcipotriene plus betamethasone helped some patients with localized sclerotic diseases. They can be irritating over genitals which require alternate-day therapy and should be used with caution in patients having compromised renal function.[]

Topical TRPM8

There is a single case report of the use of topical Sicilian in LS with relief in pruritus. Iselin is a TRPM8 receptor antagonist similar to menthol but with a higher affinity to the TRPM8 receptor.[,]

Topical avocado and soybean extracts

Borghi et al.[] suggest topical avocado and soybean extracts as alternative treatments for mild-to-moderate LS in patients wishing to avoid corticosteroids.

Results in their study provide evidence that the topical and dietary supplements exert anti-inflammatory, antifibrotic, emollient, and lenitive actions and are effective alternatives in the treatment of mild-to-moderate VLS.

Topical oxatomide

It helps relieve pruritus through its antihistamine effects, but the course of the disease is not affected.[]

Intralesional therapy

Injection of triamcinolone acetonide 20 mg directly into the thickened hypertrophic plaques of VLS once per month for 3 months[] after topical anesthetic help to minimize patient discomfort. Intralesional injection of adalimumab has also found to be beneficial.[]

Lights and Lasers

Phototherapy

Narrow-band ultraviolet B and psoralen plus ultraviolet A

A single study of ultraviolet A1 (UVA1) in seven women with VLS that had not been controlled by topical steroids[] reported initial improvement in five patients although two relapsed and the others required ongoing treatment with topical steroids. Studies have compared ultrapotent topical corticosteroids with the calcineurin inhibitors showing more efficacy of clobetasol propionate which works better compared with UVA1 phototherapy.

Photodynamic therapy using a photosensitizer

Successful treatment of VLS with photodynamic therapy (PDT) has been reported.[] In an open study of ten patients treated with two sessions of PDT, all patients reported some improvement in symptoms of VLS (itching, burning, and pain).[]

In an open study of PDT for VLS (topical 20% 5-aminolevulinic acid, argon laser light, and one to three treatments), 10 of 12 patients derived significant improvement.[] It caused significant burning although itching improved in 8 of 12 women. Another study demonstrated good symptomatic benefit in six of ten patients treated with aminolevulinic acid-PDT using a bioadhesive patch.[]

Cryotherapy

Cryotherapy of affected genital lesions after one or a series of treatments shows improvement. In one small study of 12 patients with VLS and severe intractable itch, 75% obtained symptom relief with cryotherapy.[]

Laser

Tissue-vaporizing carbon dioxide lasers, nonablative lasers such as the pulsed dye, and erbium-doped yttrium aluminum garnet lasers have been reported to benefit LS symptomatically but did not stop the disease from recurring. There is a report showing benefit in 17 out of 31 cases of untreated LS using frequencies of 5–8 MHz with focused ultrasound.[]

Systemic Therapy

Retinoids

Retinoids appear to reduce connective tissue degeneration in LS. However, the use of these agents is limited by significant and potentially harmful side effects including cheilitis, xerosis, teratogenicity, elevated liver enzymes, hypertriglyceridemia, abdominal pain, and alopecia. Isotretinoin decreases sebaceous gland size and sebum production. It may inhibit sebaceous gland differentiation and abnormal keratinization.

Oral acitretin (20–30 mg/day for 16 weeks) was effective in one randomized trial.[] Doses of 8–30 mg daily for 4 months have been used which gave benefits both in symptoms and also induce resolution of lesions. The mechanism of action of systemic retinoids in LS particularly in genital LS is not clear; mostly, it acts by downregulation of fibroblast function.

Hydroxychloroquine

It is reported to be effective in widespread genital and extragenital LSA, with conflicting results.[]

Hydroxycarbamide (hydroxyurea)

Hydroxycarbamide is an antineoplastic drug used in myeloproliferative disorders. It inhibits T-lymphocyte proliferation and gamma interferon production and has antiretroviral properties. It is used in LS in the dose of 1 g daily reducing pruritus and soreness in 6 months.[]

Cyclotron

The cyclotron is a low molecular weight interferon-inducing substance that has antiviral, immunomodulating, and anti-inflammatory effects. A prospective randomized study involved sixty patients with chronic dystrophic diseases of the vulva (45–65 years); cyclotron given intramuscular on days 1, 2, 4, 6, 8, 10, 12, 16, 20, and 23 was reported to induce rapid remission, improvement of QoL, and psychosocial function.[]

Cyclosporine

Cyclosporine has been used in only ten patients with VLS.[] Three patients had a significant improvement, five had some response, and two had no response.

Potassium Para-aminobenzoate

One report of five patients with LS at various sites and resistant to numerous other therapies documented good improvement with potassium para-aminobenzoate (4–24 g daily, in divided doses) in all five.[]

A small number of women initially have a partial response to medical treatment but have ongoing burning, irritation, and pain. In these cases, obtain cultures to exclude superinfection by Staphylococcus, Streptococcus, or Candida. The patient may have bacterial cellulitis, vulvar candidiasis, or vaginal candidiasis, which requires treatment with appropriate antibiotics or antimycotic drugs.


Diet

Twenty-three patients also received dietary supplements with Vitamin E and para-aminobenzoic acid showed improvement.[]

Surgery

VLS can be surgically excised, but mutilating gynecologic surgery is usually not recommended. The rationale behind surgical therapy is primarily to treat those patients who did not respond or responded poorly to medical treatment, secondarily to postinflammatory sequelae, and prevent the development of invasive carcinoma of the vulva.

Surgical intervention in LS is done to release a buried clitoris, to separate fused labia, or to widen a narrowed introitus in the case of pain or sexual dysfunction. V-Y advancement flap is an effective method for the reconstruction of the perineal region. This technique will allow the expansion of the vaginal orifice with good cosmetic results and rapid healing after surgery.

Introit stenosis, posterior fissuring, and scarring at the fourchette can be treated by vulvoperineoplasty.[] Since vaginal tissue is not affected by LS, part of the vaginal wall is used in the repair to prevent recurrent adhesions and fissuring at the introitus. Adhesions at the fourchette which cause dyspareunia can be extirpated. Adhesions burying the clitoris can result in the formation of painful pseudocysts. Clitoral adhesions are released with delicate knife strokes. Reformation of adhesions can be prevented by resection of a fragment of the clitoral hood in the shape of a tricorn.[]

Other points that need to be considered treating VLS are as follows:

  • Menopausal women may have symptoms related to atrophy and dryness, which will respond to topical estrogen cream and moisturizers
  • A diagnosis of superimposed vulvodynia should be considered if pain persists despite resolution of pruritus and dermal changes. It is likely that vulvodynia represents neuropathic pain, which is pain arising from abnormal neural activity secondary to disease, irritation, or injury of the nervous system that persists in the absence of ongoing disease or acute injury.
  • An allergy to the topical medication may be present. A topical steroid with a different base or consultation with an allergy specialist should be considered.

Counseling

According to the National Vulvodynia Association, women with VLS or any vulvovaginal condition experience feelings of isolation, hopelessness, depression, anxiety, anger, and low self-image. Some women are unable to continue working, any physical activity, or sexual relations.

Psychological counseling is very much needed in patients of VLS. Education relating to sexual dysfunction and dyspareunia may be required. Patients should be educated on what changes (e.g., ulceration) might indicate malignant transformation so that immediate consultation can be done.

Counseling involves self-care to be taken by patient themselves such as:

  • Avoid washing with soap or to use an emollient soap
  • Carefully dry the area after passing urine to reduce the contact of urine or using a moisturizer or soft paraffin as a barrier cream to protect skin from exposure to urine
  • If sexual intercourse is painful because of tightening at the entrance to the vagina, use of lubricants and vaginal dilators if required
  • Keep an eye on your skin. Regular self-examination is very important. If any skin change develops which does not respond to steroid creams, or there is any skin thickening, soreness, or ulceration lasting more than 2 weeks, consult without delay and get a biopsy done to rule out skin cancer.

References

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Balanitis Xerotica Obliterans – Causes, Symptoms, Treatment

Balanitis Xerotica Obliterans/Lichen Sclerosis atrophicus (LSA) or, simply, lichen sclerosis, is a disorder characterized by an atrophic epidermis, altered collagen in the superficial dermis, and an overlying white or white-blue appearance to the skin. The terms balanitis xerotica obliterans and kraurosis vulvae have been applied to genital lesions of LSA in men and women, respectively. The pathogenesis of LSA is poorly understood, but the increased incidence of LSA with other autoimmune diseases (e.g., morphea) and the presence of autoantibodies directed against extracellular matrix protein 1 suggest LSA is an autoimmune disorder.

Kraurosis vulva is a cutaneous condition characterized by atrophy and shrinkage of the skin of the vagina and vulva often accompanied by a chronic inflammatory reaction in the deeper tissues.[rx]

Lichen Sclerosis (LS) is a chronic inflammatory disease. It was first described by Hallopeau in 1881.  In 1976, the International Society for the Study of Vulvovaginal Disease adopted the term lichen sclerosis. LS is a mucocutaneous autoimmune disorder characterized by hypopigmentation and skin atrophy. It involves most commonly the genital skin, less often the extragenital sites. LS is more common in women than in men. It may cause phimosis or scarring of the vaginal introitus. The diagnosis is based on the clinical features, but it is often confirmed by biopsy. The lesions can evolve towards the destruction of anatomic structures, functional impairment, and a potential risk for malignant evolution. Thus, treatment and long term follow-up are mandatory.

Another Name

Since then, multiple names have been used to describe this condition such as leukoplakia, kraurosis vulvae, balanitis xerotica obliterans, and lichen sclerosis atrophicus.

Causes of Balanitis Xerotica Obliterans

  • LS is considered to be an autoimmune condition; however, its etiology remains unclear. Given its association with other autoimmune diseases such as alopecia areata, vitiligo, autoimmune thyroiditis, and pernicious anemia, its etiology is probably multifactorial. Further etiologic factors have been implicated such as genetic susceptibility, infectious agents such as spirochetes, sex hormone, and the Koebner phenomenon. Furthermore, recent data demonstrated a high prevalence of lichen sclerosis associated with morphea. 
  • The underlying pathogenesis of LS includes an infiltrate of activated T cells releasing interleukin 4 (IL 4) and transforming growth factor β (TGF β). So, these cytokines activate fibroblasts producing significantly altered collagen leading to fibrosis. Besides, the pathogenesis includes vascular damage by the decrease in the number of capillaries.
  • Interleukin 1 (IL 1) and interleukin one receptor antagonist (IL 1ra) may also be included in the pathogenesis of lichen sclerosis as well as an increased number of monoclonal T lymphocyte CD4 +, lymphocyte T dendritic CD1a + cells, macrophages, mast cells, and decreased number of T lymphocyte CD3 +.
  • Another hypothesis has been suggested such as an increased number of circulating IgG autoantibodies targeting extracellular matrix 1 (ECM 1) protein leading to widespread deposition of hyaline material in the dermis. 

Symptoms of Balanitis Xerotica Obliterans

  • The reduction in structural complexity of vulvar tissue commonly occurs with advancing age, which is manifest externally by drying, shriveling and white patches. Formally known as kraurosis vulvae.
  • Often asymptomatic; less commonly, itching, dyspareunia, dysuria and tenderness.
  • LSA may present at any age, from pediatric to geriatric age groups.
  • The condition is more common in women.
  • The anogenital region alone is affected in about 50% of cases, the anogenital and extragenital disease is present in about 25% of cases, and only extragenital areas are affected in about 25% of cases.
  • Extragenital LSA is common on the neck, wrists, and inframammary areas.
  • Anogenital LSA in girls and women often demonstrates an hourglass-like or figure-eight configuration that surrounds the genitalia and anal region.
  • Early LSA may be pruritic or asymptomatic.
  • Primary lesions quickly evolve into white atrophic areas, with variable telangiectasias.
  • Some cases demonstrate the surrounding brown hyperpigmentation.
  • Variable features include hyperkeratosis and follicular plugging, fissures, blister formation hemorrhage, and ulceration.
  • Phimosis is a complication in uncircumcised men, and urethral stricture may also occur.
  • Squamous cell carcinoma is a rare complication with chronic genital LSA.

Diagnosis of Balanitis Xerotica Obliterans

LS has a specific histologic pattern characterized by a band-like lymphocytic infiltrate below a zone of dermal edema and orthokeratotic hyperkeratosis. Histopathology findings vary depending on disease duration. In earlier stages, it shows vacuolar degeneration of the basal layer, hyalinization of subepithelial collagen, decreased elastic fibers in the upper dermis, and dilated blood vessels under the basement membrane. In older lesions, histology shows a reduced number of mononuclear cells and dispersed patchy islands of mononuclear cells within the hyalinized dermis.

History and Physical

LS affects most commonly the genital site and less often the extragenital area. Typical lesions begin as a sharply demarcated erythema that becomes thin, hypopigmented, ivory-white, porcelain-like, and sclerotic plaques. Plaques may become later thickened due to repeated excoriations. Itch is the main symptom and is often worse at night. Other lesions may include telangiectasias, purpura, fissures, ulcerations, and edema. Typical complaints commonly include significant pruritus, local burning sensation, pain, painful defecation. Constipation is frequent in children but rarely seen in adults. However, lesions can be asymptomatic. Genital lesions begin around the periclitoral hood. The affected area varies from a small and single area to a large area involving the entire region of the vulva, perineum, and perianus assuming a typical aspect of ‘keyhole sign.’

However, LS usually spares the vagina and cervix. In girls, LS presents commonly with irritation and soreness although it can mimic sexual abuse. However, LS and sexual abuse can coexist. Visual lichen sclerosis exhibits the Koebner phenomenon at sites of trauma; sexual abuse can aggravate lesions of LS.  Male genital LS (boys and men) occurs in the foreskin, glans penis, and the coronal sulcus penis. Extragenital lesions occur on any part of the skin and usually asymptomatic. The most commonly involved areas are inframammary areas, neck, wrists, thighs, upper back, and shoulders. The involvement of the oral mucosa appears clinically as bluish-white papules on the buccal mucosa or under the tongue.

Evaluation

The diagnosis is based on taking a careful history including the autoimmune diseases in the patient and family, the examination of the mucosas, extragenital skin, and completed by a gynecological exam. Thus, the diagnosis of LS is usually clinical. But in some cases, a biopsy can be performed. However, atypical histology does not rule out the diagnosis.

Biopsies should be performed in case of:

  • Atypical clinical presentation
  • Suspected malignancy
  • Nonresponse, after an appropriate duration, to recommended first-line treatment

The workup of the condition should include investigation of thyroid function, and according to symptoms investigation of the other autoimmune diseases.

Treatment of Balanitis Xerotica Obliterans

The aims of the treatment are relief of the symptoms, stopping the atrophy, prevention of scar formation, and anatomical distortion, as well as malignant transformation. The therapy includes general care, topical treatments, systemic treatments, and surgical procedures. It is fundamental to inform the patient to avoid the use of irritating products such as soap and to prefer emollients to break the itch-stretch cycle.

For genital LS, the gold standard treatment is three months application of high potency topical steroids (clobetasol propionate). Second-line therapies include topical calcineurin inhibitors and imiquimod. In men, early circumcision may be recommended. Surgery is indicated only for the treatment of complications associated with lichen sclerosus. For extragenital LS, therapeutic modalities are limited and include phototherapy, ultrapotent topical steroids, tacrolimus ointment 0, 1%, and systemic steroids or methotrexate. Follow-up examinations must be kept indefinitely. 

There is no definitive cure for LS.[] Behavior change, such as good hygiene and minimizing scratching, is an important part of treatment,[] so a more realistic goal is to control pruritus rather than resolution of the lesion. Various treatment modalities have been tried with varied results.


Topical Treatment

Hormonal therapy

  • Estrogen is an effective treatment for postmenopausal vulvovaginal atrophy and should be considered in women with dyspareunia, labial fusion, or epidermal thinning due to estrogen deficiency but not as primary therapy of LS. Moisturizers and estrogens help in dryness and atrophy
  • Topical testosterone (2%) and progesterone (2%) were mainstays of treatment for decades and were reported to induce remission of LS. It acts by reducing inflammation and helps to relieve symptoms and in some cases, resolves the lesion, but androgenic side effects such as clitoral enlargement, hirsutism, acne vulgaris, and amenorrhea are common.

Topical steroids

VLS responds to ultrapotent topical corticosteroids, i.e, clobetasol propionate or mometasone furoate, though the clinical appearance does not reverse, the patient gets symptomatic relief and it prevents scarring. Clobetasol or clobetasol propionate 0.05% ointment is a Class I superpotent topical steroid which suppresses mitosis, increases the synthesis of proteins, decreases inflammation, and causes vasoconstriction. It is given daily at night for 6–12 weeks and then one to three times per week for maintenance.

Females should be evaluated regularly to see for skin atrophy or any malignant change. Prepubertal LS in girls may resolve spontaneously although some of them may suffer from various types of vulvodynia in adulthood.

Topical calcineurin inhibitors

Tacrolimus (0.1%) and pimecrolimus (1%) have a role as maintenance therapy but not as effective as potent topical corticosteroids and may be useful as alternative treatment options. It reduces itching and inflammation by suppressing the release of cytokines from T-cells and inhibits transcription for genes that encode interleukin (IL)-3, IL-4, IL-5, granulocyte-macrophage colony-stimulating factor, and tumor necrosis factor-alpha, which are involved in the early stages of T-cell activation. It also inhibits the release of preformed mediators from skin mast cells and basophils and downregulates the expression of FCeRI on Langerhans cells. Pimecrolimus is derived from ascomycin, a natural substance produced by Streptomyces hygroscopicus var. ascomycetous which selectively inhibits the production and release of inflammatory cytokines from activated T-cells by binding to cytosolic immunophilin receptor macrophilia-12. The resulting complex inhibits phosphatase calcineurin, thus blocking T-cell activation and cytokine release.[]

Topical retinoids

Topical tretinoin (0.025%) and tazarotene (0.01%) have off-label indications in LS. They act by downregulation of fibroblast function. Especially in genital areas, short-contact therapy is used in which the gel is initially applied for 15 min and washed off. Every 2–3 weeks, application time is increased by 15 min until therapeutic effects are noted.

In an observational series, topical use of tretinoin improved the symptoms, gross appearance, and histopathologic features of LS with minimal side effects.[]

Vitamin D analogs

Calcitriol, calcipotriene, and calcipotriene plus betamethasone helped some patients with localized sclerotic diseases. They can be irritating over genitals which require alternate-day therapy and should be used with caution in patients having compromised renal function.[]

Topical TRPM8

There is a single case report of the use of topical Sicilian in LS with relief in pruritus. Iselin is a TRPM8 receptor antagonist similar to menthol but with a higher affinity to the TRPM8 receptor.[,]

Topical avocado and soybean extracts

Borghi et al.[] suggest topical avocado and soybean extracts as alternative treatments for mild-to-moderate LS in patients wishing to avoid corticosteroids.

Results in their study provide evidence that the topical and dietary supplements exert anti-inflammatory, antifibrotic, emollient, and lenitive actions and are effective alternatives in the treatment of mild-to-moderate VLS.

Topical oxatomide

It helps relieve pruritus through its antihistamine effects, but the course of the disease is not affected.[]

Intralesional therapy

Injection of triamcinolone acetonide 20 mg directly into the thickened hypertrophic plaques of VLS once per month for 3 months[] after topical anesthetic help to minimize patient discomfort. Intralesional injection of adalimumab has also found to be beneficial.[]

Lights and Lasers

Phototherapy

Narrow-band ultraviolet B and psoralen plus ultraviolet A

A single study of ultraviolet A1 (UVA1) in seven women with VLS that had not been controlled by topical steroids[] reported initial improvement in five patients although two relapsed and the others required ongoing treatment with topical steroids. Studies have compared ultrapotent topical corticosteroids with the calcineurin inhibitors showing more efficacy of clobetasol propionate which works better compared with UVA1 phototherapy.

Photodynamic therapy using a photosensitizer

Successful treatment of VLS with photodynamic therapy (PDT) has been reported.[] In an open study of ten patients treated with two sessions of PDT, all patients reported some improvement in symptoms of VLS (itching, burning, and pain).[]

In an open study of PDT for VLS (topical 20% 5-aminolevulinic acid, argon laser light, and one to three treatments), 10 of 12 patients derived significant improvement.[] It caused significant burning although itching improved in 8 of 12 women. Another study demonstrated good symptomatic benefit in six of ten patients treated with aminolevulinic acid-PDT using a bioadhesive patch.[]

Cryotherapy

Cryotherapy of affected genital lesions after one or a series of treatments shows improvement. In one small study of 12 patients with VLS and severe intractable itch, 75% obtained symptom relief with cryotherapy.[]

Laser

Tissue-vaporizing carbon dioxide lasers, nonablative lasers such as the pulsed dye, and erbium-doped yttrium aluminum garnet lasers have been reported to benefit LS symptomatically but did not stop the disease from recurring. There is a report showing benefit in 17 out of 31 cases of untreated LS using frequencies of 5–8 MHz with focused ultrasound.[]

Systemic Therapy

Retinoids

Retinoids appear to reduce connective tissue degeneration in LS. However, the use of these agents is limited by significant and potentially harmful side effects including cheilitis, xerosis, teratogenicity, elevated liver enzymes, hypertriglyceridemia, abdominal pain, and alopecia. Isotretinoin decreases sebaceous gland size and sebum production. It may inhibit sebaceous gland differentiation and abnormal keratinization.

Oral acitretin (20–30 mg/day for 16 weeks) was effective in one randomized trial.[] Doses of 8–30 mg daily for 4 months have been used which gave benefits both in symptoms and also induce resolution of lesions. The mechanism of action of systemic retinoids in LS particularly in genital LS is not clear; mostly, it acts by downregulation of fibroblast function.

Hydroxychloroquine

It is reported to be effective in widespread genital and extragenital LSA, with conflicting results.[]

Hydroxycarbamide (hydroxyurea)

Hydroxycarbamide is an antineoplastic drug used in myeloproliferative disorders. It inhibits T-lymphocyte proliferation and gamma interferon production and has antiretroviral properties. It is used in LS in the dose of 1 g daily reducing pruritus and soreness in 6 months.[]

Cyclotron

The cyclotron is a low molecular weight interferon-inducing substance that has antiviral, immunomodulating, and anti-inflammatory effects. A prospective randomized study involved sixty patients with chronic dystrophic diseases of the vulva (45–65 years); cyclotron gave intramuscular on days 1, 2, 4, 6, 8, 10, 12, 16, 20, and 23 were reported to induce rapid remission, improvement of QoL, and psychosocial function.[]

Cyclosporine

Cyclosporine has been used in only ten patients with VLS.[] Three patients had a significant improvement, five had some response, and two had no response.

Potassium Para-aminobenzoate

One report of five patients with LS at various sites and resistant to numerous other therapies documented good improvement with potassium para-aminobenzoate (4–24 g daily, in divided doses) in all five.[]

A small number of women initially have a partial response to medical treatment but have ongoing burning, irritation, and pain. In these cases, obtain cultures to exclude superinfection by Staphylococcus, Streptococcus, or Candida. The patient may have bacterial cellulitis, vulvar candidiasis, or vaginal candidiasis, which requires treatment with appropriate antibiotics or antimycotic drugs.


Diet

Twenty-three patients also received dietary supplements with Vitamin E and para-aminobenzoic acid showed improvement.[]

Surgery

VLS can be surgically excised, but mutilating gynecologic surgery is usually not recommended. The rationale behind surgical therapy is primarily to treat those patients who did not respond or responded poorly to medical treatment, secondarily to postinflammatory sequelae, and prevent the development of invasive carcinoma of the vulva.

Surgical intervention in LS is done to release a buried clitoris, to separate fused labia, or to widen a narrowed introitus in the case of pain or sexual dysfunction. V-Y advancement flap is an effective method for the reconstruction of the perineal region. This technique will allow the expansion of the vaginal orifice with good cosmetic results and rapid healing after surgery.

Introit stenosis, posterior fissuring, and scarring at the fourchette can be treated by vulvoperineoplasty.[] Since vaginal tissue is not affected by LS, part of the vaginal wall is used in the repair to prevent recurrent adhesions and fissuring at the introitus. Adhesions at the fourchette which cause dyspareunia can be extirpated. Adhesions burying the clitoris can result in the formation of painful pseudocysts. Clitoral adhesions are released with delicate knife strokes. Reformation of adhesions can be prevented by resection of a fragment of the clitoral hood in the shape of a tricorn.[]

Other points that need to be considered treating VLS are as follows:

  • Menopausal women may have symptoms related to atrophy and dryness, which will respond to topical estrogen cream and moisturizers
  • A diagnosis of superimposed vulvodynia should be considered if pain persists despite resolution of pruritus and dermal changes. It is likely that vulvodynia represents neuropathic pain, which is pain arising from abnormal neural activity secondary to disease, irritation, or injury of the nervous system that persists in the absence of ongoing disease or acute injury.
  • An allergy to the topical medication may be present. A topical steroid with a different base or consultation with an allergy specialist should be considered.

Counseling

According to the National Vulvodynia Association, women with VLS or any vulvovaginal condition experience feelings of isolation, hopelessness, depression, anxiety, anger, and low self-image. Some women are unable to continue working, any physical activity, or sexual relations.

Psychological counseling is very much needed in patients of VLS. Education relating to sexual dysfunction and dyspareunia may be required. Patients should be educated on what changes (e.g., ulceration) might indicate malignant transformation so that immediate consultation can be done.

Counseling involves self-care to be taken by patient themselves such as:

  • Avoid washing with soap or to use an emollient soap
  • Carefully dry the area after passing urine to reduce the contact of urine or using a moisturizer or soft paraffin as a barrier cream to protect skin from exposure to urine
  • If sexual intercourse is painful because of tightening at the entrance to the vagina, use of lubricants and vaginal dilators if required
  • Keep an eye on your skin. Regular self-examination is very important. If any skin change develops which does not respond to steroid creams, or there is any skin thickening, soreness, or ulceration lasting more than 2 weeks, consult without delay and get a biopsy done to rule out skin cancer.

References

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Hypopigmentation Skin Atrophy – Symptoms, Treatment

Hypopigmentation Skin Atrophy/Lichen Sclerosis atrophicus (LSA) or, simply, lichen sclerosis, is a disorder characterized by an atrophic epidermis, altered collagen in the superficial dermis, and an overlying white or white-blue appearance to the skin. The terms balanitis xerotica obliterans and kraurosis vulvae have been applied to genital lesions of LSA in men and women, respectively. The pathogenesis of LSA is poorly understood, but the increased incidence of LSA with other autoimmune diseases (e.g., morphea) and the presence of autoantibodies directed against extracellular matrix protein 1 suggest LSA is an autoimmune disorder.

Kraurosis vulva is a cutaneous condition characterized by atrophy and shrinkage of the skin of the vagina and vulva often accompanied by a chronic inflammatory reaction in the deeper tissues.[rx]

Lichen Sclerosis (LS) is a chronic inflammatory disease. It was first described by Hallopeau in 1881.  In 1976, the International Society for the Study of Vulvovaginal Disease adopted the term lichen sclerosis. LS is a mucocutaneous autoimmune disorder characterized by hypopigmentation and skin atrophy. It involves most commonly the genital skin, less often the extragenital sites. LS is more common in women than in men. It may cause phimosis or scarring of the vaginal introitus. The diagnosis is based on the clinical features, but it is often confirmed by biopsy. The lesions can evolve towards the destruction of anatomic structures, functional impairment, and a potential risk for malignant evolution. Thus, treatment and long term follow-up are mandatory.

Another Name

Since then, multiple names have been used to describe this condition such as leukoplakia, kraurosis vulvae, balanitis xerotica obliterans, and lichen sclerosis atrophicus.

Causes of Hypopigmentation Skin Atrophy

  • LS is considered to be an autoimmune condition; however, its etiology remains unclear. Given its association with other autoimmune diseases such as alopecia areata, vitiligo, autoimmune thyroiditis, and pernicious anemia, its etiology is probably multifactorial. Further etiologic factors have been implicated such as genetic susceptibility, infectious agents such as spirochetes, sex hormone, and the Koebner phenomenon. Furthermore, recent data demonstrated a high prevalence of lichen sclerosis associated with morphea. 
  • The underlying pathogenesis of LS includes an infiltrate of activated T cells releasing interleukin 4 (IL 4) and transforming growth factor β (TGF β). So, these cytokines activate fibroblasts producing significantly altered collagen leading to fibrosis. Besides, the pathogenesis includes vascular damage by the decrease in the number of capillaries.
  • Interleukin 1 (IL 1) and interleukin one receptor antagonist (IL 1ra) may also be included in the pathogenesis of lichen sclerosis as well as an increased number of monoclonal T lymphocyte CD4 +, lymphocyte T dendritic CD1a + cells, macrophages, mast cells, and decreased number of T lymphocyte CD3 +.
  • Another hypothesis has been suggested such as an increased number of circulating IgG autoantibodies targeting extracellular matrix 1 (ECM 1) protein leading to widespread deposition of hyaline material in the dermis. 

Symptoms of Hypopigmentation Skin Atrophy

  • The reduction in structural complexity of vulvar tissue commonly occurs with advancing age, which is manifest externally by drying, shriveling and white patches. Formally known as kraurosis vulvae.
  • Often asymptomatic; less commonly, itching, dyspareunia, dysuria and tenderness.
  • LSA may present at any age, from pediatric to geriatric age groups.
  • The condition is more common in women.
  • The anogenital region alone is affected in about 50% of cases, the anogenital and extragenital disease is present in about 25% of cases, and only extragenital areas are affected in about 25% of cases.
  • Extragenital LSA is common on the neck, wrists, and inframammary areas.
  • Anogenital LSA in girls and women often demonstrates an hourglass-like or figure-eight configuration that surrounds the genitalia and anal region.
  • Early LSA may be pruritic or asymptomatic.
  • Primary lesions quickly evolve into white atrophic areas, with variable telangiectasias.
  • Some cases demonstrate the surrounding brown hyperpigmentation.
  • Variable features include hyperkeratosis and follicular plugging, fissures, blister formation hemorrhage, and ulceration.
  • Phimosis is a complication in uncircumcised men, and urethral stricture may also occur.
  • Squamous cell carcinoma is a rare complication with chronic genital LSA.

Diagnosis of Hypopigmentation Skin Atrophy

LS has a specific histologic pattern characterized by a band-like lymphocytic infiltrate below a zone of dermal edema and orthokeratotic hyperkeratosis. Histopathology findings vary depending on disease duration. In earlier stages, it shows vacuolar degeneration of the basal layer, hyalinization of subepithelial collagen, decreased elastic fibers in the upper dermis, and dilated blood vessels under the basement membrane. In older lesions, histology shows a reduced number of mononuclear cells and dispersed patchy islands of mononuclear cells within the hyalinized dermis.

History and Physical

LS affects most commonly the genital site and less often the extragenital area. Typical lesions begin as a sharply demarcated erythema that becomes thin, hypopigmented, ivory-white, porcelain-like, and sclerotic plaques. Plaques may become later thickened due to repeated excoriations. Itch is the main symptom and is often worse at night. Other lesions may include telangiectasias, purpura, fissures, ulcerations, and edema. Typical complaints commonly include significant pruritus, local burning sensation, pain, painful defecation. Constipation is frequent in children but rarely seen in adults. However, lesions can be asymptomatic. Genital lesions begin around the periclitoral hood. The affected area varies from a small and single area to a large area involving the entire region of the vulva, perineum, and perianus assuming a typical aspect of ‘keyhole sign.’

However, LS usually spares the vagina and cervix. In girls, LS presents commonly with irritation and soreness although it can mimic sexual abuse. However, LS and sexual abuse can coexist. Visual lichen sclerosis exhibits the Koebner phenomenon at sites of trauma; sexual abuse can aggravate lesions of LS.  Male genital LS (boys and men) occurs in the foreskin, glans penis, and the coronal sulcus penis. Extragenital lesions occur on any part of the skin and usually asymptomatic. The most commonly involved areas are inframammary areas, neck, wrists, thighs, upper back, and shoulders. The involvement of the oral mucosa appears clinically as bluish-white papules on the buccal mucosa or under the tongue.

Evaluation

The diagnosis is based on taking a careful history including the autoimmune diseases in the patient and family, the examination of the mucosas, extragenital skin, and completed by a gynecological exam. Thus, the diagnosis of LS is usually clinical. But in some cases, a biopsy can be performed. However, atypical histology does not rule out the diagnosis.

Biopsies should be performed in case of:

  • Atypical clinical presentation
  • Suspected malignancy
  • Nonresponse, after an appropriate duration, to recommended first-line treatment

The workup of the condition should include investigation of thyroid function, and according to symptoms investigation of the other autoimmune diseases.

Treatment of Hypopigmentation Skin Atrophy

The aims of the treatment are relief of the symptoms, stopping the atrophy, prevention of scar formation, and anatomical distortion, as well as malignant transformation. The therapy includes general care, topical treatments, systemic treatments, and surgical procedures. It is fundamental to inform the patient to avoid the use of irritating products such as soap and to prefer emollients to break the itch-stretch cycle.

For genital LS, the gold standard treatment is three months application of high potency topical steroids (clobetasol propionate). Second-line therapies include topical calcineurin inhibitors and imiquimod. In men, early circumcision may be recommended. Surgery is indicated only for the treatment of complications associated with lichen sclerosus. For extragenital LS, therapeutic modalities are limited and include phototherapy, ultrapotent topical steroids, tacrolimus ointment 0, 1%, and systemic steroids or methotrexate. Follow-up examinations must be kept indefinitely. 

There is no definitive cure for LS.[] Behavior change, such as good hygiene and minimizing scratching, is an important part of treatment,[] so a more realistic goal is to control pruritus rather than resolution of the lesion. Various treatment modalities have been tried with varied results.


Topical Treatment

Hormonal therapy

  • Estrogen is an effective treatment for postmenopausal vulvovaginal atrophy and should be considered in women with dyspareunia, labial fusion, or epidermal thinning due to estrogen deficiency but not as primary therapy of LS. Moisturizers and estrogens help in dryness and atrophy
  • Topical testosterone (2%) and progesterone (2%) were mainstays of treatment for decades and were reported to induce remission of LS. It acts by reducing inflammation and helps to relieve symptoms and in some cases, resolves the lesion, but androgenic side effects such as clitoral enlargement, hirsutism, acne vulgaris, and amenorrhea are common.

Topical steroids

VLS responds to ultrapotent topical corticosteroids, i.e, clobetasol propionate or mometasone furoate, though the clinical appearance does not reverse, the patient gets symptomatic relief and it prevents scarring. Clobetasol or clobetasol propionate 0.05% ointment is a Class I superpotent topical steroid which suppresses mitosis, increases the synthesis of proteins, decreases inflammation, and causes vasoconstriction. It is given daily at night for 6–12 weeks and then one to three times per week for maintenance.

Females should be evaluated regularly to see for skin atrophy or any malignant change. Prepubertal LS in girls may resolve spontaneously although some of them may suffer from various types of vulvodynia in adulthood.

Topical calcineurin inhibitors

Tacrolimus (0.1%) and pimecrolimus (1%) have a role as maintenance therapy but not as effective as potent topical corticosteroids and may be useful as alternative treatment options. It reduces itching and inflammation by suppressing the release of cytokines from T-cells and inhibits transcription for genes that encode interleukin (IL)-3, IL-4, IL-5, granulocyte-macrophage colony-stimulating factor, and tumor necrosis factor-alpha, which are involved in the early stages of T-cell activation. It also inhibits the release of preformed mediators from skin mast cells and basophils and downregulates the expression of FCeRI on Langerhans cells. Pimecrolimus is derived from ascomycin, a natural substance produced by Streptomyces hygroscopicus var. ascomycetous which selectively inhibits the production and release of inflammatory cytokines from activated T-cells by binding to cytosolic immunophilin receptor macrophilia-12. The resulting complex inhibits phosphatase calcineurin, thus blocking T-cell activation and cytokine release.[]

Topical retinoids

Topical tretinoin (0.025%) and tazarotene (0.01%) have off-label indications in LS. They act by downregulation of fibroblast function. Especially in genital areas, short-contact therapy is used in which the gel is initially applied for 15 min and washed off. Every 2–3 weeks, application time is increased by 15 min until therapeutic effects are noted.

In an observational series, topical use of tretinoin improved the symptoms, gross appearance, and histopathologic features of LS with minimal side effects.[]

Vitamin D analogs

Calcitriol, calcipotriene, and calcipotriene plus betamethasone helped some patients with localized sclerotic diseases. They can be irritating over genitals which require alternate-day therapy and should be used with caution in patients having compromised renal function.[]

Topical TRPM8

There is a single case report of the use of topical Sicilian in LS with relief in pruritus. Iselin is a TRPM8 receptor antagonist similar to menthol but with a higher affinity to the TRPM8 receptor.[,]

Topical avocado and soybean extracts

Borghi et al.[] suggest topical avocado and soybean extracts as alternative treatments for mild-to-moderate LS in patients wishing to avoid corticosteroids.

Results in their study provide evidence that the topical and dietary supplements exert anti-inflammatory, antifibrotic, emollient, and lenitive actions and are effective alternatives in the treatment of mild-to-moderate VLS.

Topical oxatomide

It helps relieve pruritus through its antihistamine effects, but the course of the disease is not affected.[]

Intralesional therapy

Injection of triamcinolone acetonide 20 mg directly into the thickened hypertrophic plaques of VLS once per month for 3 months[] after topical anesthetic help to minimize patient discomfort. Intralesional injection of adalimumab has also found to be beneficial.[]

Lights and Lasers

Phototherapy

Narrow-band ultraviolet B and psoralen plus ultraviolet A

A single study of ultraviolet A1 (UVA1) in seven women with VLS that had not been controlled by topical steroids[] reported initial improvement in five patients although two relapsed and the others required ongoing treatment with topical steroids. Studies have compared ultrapotent topical corticosteroids with the calcineurin inhibitors showing more efficacy of clobetasol propionate which works better compared with UVA1 phototherapy.

Photodynamic therapy using a photosensitizer

Successful treatment of VLS with photodynamic therapy (PDT) has been reported.[] In an open study of ten patients treated with two sessions of PDT, all patients reported some improvement in symptoms of VLS (itching, burning, and pain).[]

In an open study of PDT for VLS (topical 20% 5-aminolevulinic acid, argon laser light, and one to three treatments), 10 of 12 patients derived significant improvement.[] It caused significant burning although itching improved in 8 of 12 women. Another study demonstrated good symptomatic benefit in six of ten patients treated with aminolevulinic acid-PDT using a bioadhesive patch.[]

Cryotherapy

Cryotherapy of affected genital lesions after one or a series of treatments shows improvement. In one small study of 12 patients with VLS and severe intractable itch, 75% obtained symptom relief with cryotherapy.[]

Laser

Tissue-vaporizing carbon dioxide lasers, nonablative lasers such as the pulsed dye, and erbium-doped yttrium aluminum garnet lasers have been reported to benefit LS symptomatically but did not stop the disease from recurring. There is a report showing benefit in 17 out of 31 cases of untreated LS using frequencies of 5–8 MHz with focused ultrasound.[]

Systemic Therapy

Retinoids

Retinoids appear to reduce connective tissue degeneration in LS. However, the use of these agents is limited by significant and potentially harmful side effects including cheilitis, xerosis, teratogenicity, elevated liver enzymes, hypertriglyceridemia, abdominal pain, and alopecia. Isotretinoin decreases sebaceous gland size and sebum production. It may inhibit sebaceous gland differentiation and abnormal keratinization.

Oral acitretin (20–30 mg/day for 16 weeks) was effective in one randomized trial.[] Doses of 8–30 mg daily for 4 months have been used which gave benefits both in symptoms and also induce resolution of lesions. The mechanism of action of systemic retinoids in LS particularly in genital LS is not clear; mostly, it acts by downregulation of fibroblast function.

Hydroxychloroquine

It is reported to be effective in widespread genital and extragenital LSA, with conflicting results.[]

Hydroxycarbamide (hydroxyurea)

Hydroxycarbamide is an antineoplastic drug used in myeloproliferative disorders. It inhibits T-lymphocyte proliferation and gamma interferon production and has antiretroviral properties. It is used in LS in the dose of 1 g daily reducing pruritus and soreness in 6 months.[]

Cyclotron

The cyclotron is a low molecular weight interferon-inducing substance that has antiviral, immunomodulating, and anti-inflammatory effects. A prospective randomized study involved sixty patients with chronic dystrophic diseases of the vulva (45–65 years); cyclotron gave intramuscular on days 1, 2, 4, 6, 8, 10, 12, 16, 20, and 23 were reported to induce rapid remission, improvement of QoL, and psychosocial function.[]

Cyclosporine

Cyclosporine has been used in only ten patients with VLS.[] Three patients had a significant improvement, five had some response, and two had no response.

Potassium Para-aminobenzoate

One report of five patients with LS at various sites and resistant to numerous other therapies documented good improvement with potassium para-aminobenzoate (4–24 g daily, in divided doses) in all five.[]

A small number of women initially have a partial response to medical treatment but have ongoing burning, irritation, and pain. In these cases, obtain cultures to exclude superinfection by Staphylococcus, Streptococcus, or Candida. The patient may have bacterial cellulitis, vulvar candidiasis, or vaginal candidiasis, which requires treatment with appropriate antibiotics or antimycotic drugs.


Diet

Twenty-three patients also received dietary supplements with Vitamin E and para-aminobenzoic acid showed improvement.[]

Surgery

VLS can be surgically excised, but mutilating gynecologic surgery is usually not recommended. The rationale behind surgical therapy is primarily to treat those patients who did not respond or responded poorly to medical treatment, secondarily to postinflammatory sequelae, and prevent the development of invasive carcinoma of the vulva.

Surgical intervention in LS is done to release a buried clitoris, to separate fused labia, or to widen a narrowed introitus in the case of pain or sexual dysfunction. V-Y advancement flap is an effective method for the reconstruction of the perineal region. This technique will allow the expansion of the vaginal orifice with good cosmetic results and rapid healing after surgery.

Introit stenosis, posterior fissuring, and scarring at the fourchette can be treated by vulvoperineoplasty.[] Since vaginal tissue is not affected by LS, part of the vaginal wall is used in the repair to prevent recurrent adhesions and fissuring at the introitus. Adhesions at the fourchette which cause dyspareunia can be extirpated. Adhesions burying the clitoris can result in the formation of painful pseudocysts. Clitoral adhesions are released with delicate knife strokes. Reformation of adhesions can be prevented by resection of a fragment of the clitoral hood in the shape of a tricorn.[]

Other points that need to be considered treating VLS are as follows:

  • Menopausal women may have symptoms related to atrophy and dryness, which will respond to topical estrogen cream and moisturizers
  • A diagnosis of superimposed vulvodynia should be considered if pain persists despite resolution of pruritus and dermal changes. It is likely that vulvodynia represents neuropathic pain, which is pain arising from abnormal neural activity secondary to disease, irritation, or injury of the nervous system that persists in the absence of ongoing disease or acute injury.
  • An allergy to the topical medication may be present. A topical steroid with a different base or consultation with an allergy specialist should be considered.

Counseling

According to the National Vulvodynia Association, women with VLS or any vulvovaginal condition experience feelings of isolation, hopelessness, depression, anxiety, anger, and low self-image. Some women are unable to continue working, any physical activity, or sexual relations.

Psychological counseling is very much needed in patients of VLS. Education relating to sexual dysfunction and dyspareunia may be required. Patients should be educated on what changes (e.g., ulceration) might indicate malignant transformation so that immediate consultation can be done.

Counseling involves self-care to be taken by patient themselves such as:

  • Avoid washing with soap or to use an emollient soap
  • Carefully dry the area after passing urine to reduce the contact of urine or using a moisturizer or soft paraffin as a barrier cream to protect skin from exposure to urine
  • If sexual intercourse is painful because of tightening at the entrance to the vagina, use of lubricants and vaginal dilators if required
  • Keep an eye on your skin. Regular self-examination is very important. If any skin change develops which does not respond to steroid creams, or there is any skin thickening, soreness, or ulceration lasting more than 2 weeks, consult without delay and get a biopsy done to rule out skin cancer.

References

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12 Yoga Poses for Neck Pain

12 Yoga Poses for Neck Pain/Neck pain is extremely common and may be caused by several factors. These include daily activities that involve repetitive forward movement patterns, poor posture, or the habit of holding your head in one position.

It doesn’t take a lot to develop pain in this area of your body, and it’s easy for that pain to extend to your shoulders and back. Neck pain can lead to headaches and even injury.

Practicing yoga is an excellent way to get rid of neck pain. At least one study found yoga to provide pain relief and functional improvements for people who did yoga for nine weeks. Through the practice, you can learn to release any tension you’re holding in your body.

Yoga may be useful in treating even chronic neck pain.

Poses for relief

Here are some of the yoga poses that may be beneficial in relieving neck pain.

Standing forward bend pose

  • Come into a standing position with your feet under your hips.
  • Lengthen your body as you fold your upper body forward, keeping a slight bend in your knees.
  • Bring your hands to your legs, a block, or the floor.
  • Tuck your chin into your chest, and let your head and neck fully relax.
  • You can gently shake your head from side to side, front to back or make gentle circles. This helps to release tension in your neck and shoulders.
  • Hold this position for at least 1 minute.
  • Bring your arms and head up last as you roll your spine up to standing.

Warrior II pose

Warrior II allows you to open and strengthen your chest and shoulders to support your neck.

  • From standing, bring your left foot back with your toes facing out to the left at a slight angle.
  • Bring your right foot forward.
  • The inside of your left foot should be in line with your right foot.
  • Bring up your arms until they’re parallel to the floor, with your palms facing down.
  • Bend your right knee, being careful not to extend your knee further forward than your ankle.
  • Press into both feet as you extend up through your spine.
  • Look out past your right fingertips.
  • Remain in this pose for 30 seconds.
  • Then do the opposite side.

Extended triangle pose

Triangle pose helps to relieve pain and tension in your neck, shoulders, and upper back.

  • Jump, step, or walk your feet apart so that they’re wider than your hips.
  • Turn your right toes forward and your left toes out at an angle.
  • Bring your arms up so they’re parallel to the floor with your palms facing down.
  • Reach forward with your right arm as you hinge at your right hip.
  • From here, lower your right arm and lift your left arm up toward the ceiling.
  • Turn your gaze in any direction or you can do gentle neck rotations looking up and down.
  • Remain in this pose for 30 seconds.
  • Then do it on the other side.

Cat cow pose

Flexing and extending the neck allows for the release of tension.

  • Begin on all fours with your hands under your shoulders and your knees under your hips.
  • On an inhale, allow your belly to fill with air and lower toward the floor.
  • Look up at the ceiling as you let your head drop back slightly.
  • Keep your head here or lower your chin slightly.
  • On an exhale, turn to look over your right shoulder.
  • Hold your gaze here for a few moments and then return to center.
  • Exhale to look over your left shoulder.
  • Hold that position before returning to the center.
  • From here, tuck your chin into your chest as you round your spine.
  • Hold this position, letting your head hang down.
  • Shake your head from side to side and forward and backward.
  • After these variations, continue the fluid motion of cat-cow pose for at least 1 minute.

Thread the needle pose

This pose helps to relieve tension in your neck, shoulders, and back.

  • Start on all fours with your wrists under your shoulders and your knees under your hips.
  • Lift your right hand and move it over to the left along the floor with your palm facing up.
  • Press your left hand into the floor for support as your rest your body on your right shoulder and look over to the left.
  • Remain in this position for 30 seconds.
  • Slowly release, sink back into Child’s Pose (see below) for a few breaths, and repeat on the other side.

Cow face pose

Cow face pose helps to stretch and open your chest and shoulders.

  • Come into a comfortable seated position.
  • Raise your left elbow and bend your arm so your hand comes to your back.
  • Use your right hand to gently pull your left elbow over to the right, or bring your right hand up to reach and hold your left hand.
  • Remain in this pose for 30 seconds.
  • Then do it on the other side.

Half lord of the fishes pose

This twist stretches the spine, shoulders, and hips.

  • From a seated position, bring your right foot along the floor to the outside of your left hip.
  • Bend your left knee and cross it over your right leg so that your left foot is “rooted” into the floor to the outside of your right thigh.
  • Lengthen your spine and then twist your upper body to the left.
  • Place your left hand on the floor behind your buttocks.
  • Bring your right arm to the outside of your left leg.
  • Turn your head to look over either shoulder or do gentle neck movements forward and backward.
  • Stay in this pose for 1 minute.
  • Then do it on the opposite side.

Sphinx pose

Sphinx pose strengthens your spine and stretches your shoulders.

  • Lie down flat on your stomach with your elbows under your shoulders, pressing into your palms and forearms.
  • Tighten your lower back, buttocks, and thighs to support you as you lift your upper torso and head.
  • Keep your gaze straight ahead and make sure you’re lengthening your spine.
  • Hold this pose for 2 minutes.

Extended puppy pose

This pose is great for relieving stress and stretching your back and shoulders.

  • Begin on all fours with your wrists directly below your shoulders and your knees directly under your hips.
  • Walk your hands forward slightly and lift your heels to come up onto your toes.
  • Slowly bring your buttocks down toward your heels, stopping halfway down.
  • Engage your arms and keep your elbows lifted.
  • Rest your forehead on the floor or a blanket.
  • Allow your neck to fully relax.
  • Keep your lower back slightly bent as you press into your palms, stretching your arms, and drawing your hips down toward your heels.
  • Hold for 1 minute.

Child’s pose

A child’s pose can help to relieve neck pain as well as a headache.

  • From a kneeling position, sit back on your heels and bring your knees to a comfortable position.
  • Lengthen your spine and walk your hands in front of you, hinging your hips so that you can fold forward.
  • Keep your arms extended in front of you to support your neck, or you can stack your hands and rest your head on them. This may help to relieve headache tension. If it’s comfortable, bring your arms back to lie along the side of your body.
  • Breathe deeply and focus on letting go of any tension or tightness you’re holding in your body.
  • Rest in this pose for a few minutes.

Legs-up-the-wall pose

This restorative pose has amazing healing potential and can help to relieve tension in your back, shoulders, and neck.

  • From a seated position, scoot forward on your hips toward a wall. When you are close to the wall, lie back and swing your legs up and against the wall.
  • You can place a folded blanket or pillow under your hips for support.
  • Bring your arms into any comfortable position.
  • You may wish to gently massage your face, neck, and shoulders.
  • Stay in this pose for up to 20 minutes.

Corpse pose

Allow yourself time at the end of your practice to relax in a corpse pose. Focus on letting go of any remaining stress and tension in your body.

  • Lie down on your back with your feet a little wider than your hips and your toes splayed out to the side.
  • Rest your arms alongside your body with your palms facing up.
  • Adjust your body so that your head, neck, and spine are aligned.
  • Focus on breathing deeply and releasing any tightness in your body.
  • Remain in this pose for at least 5 minutes.

General tips

Since these poses are designed to treat a specific ailment, it’s important that you follow these tips:

  • Remember that your body changes from day today. Make adjustments to your practice as necessary and avoid poses that cause pain or discomfort.
  • Allow your breath to guide your movement so that you’re moving slowly and with fluidity.
  • Only go to your edge — don’t push or force yourself into any position.
  • If you’re new to yoga, try to take a few classes at a local studio. If this isn’t possible, you can do guided classes online.
  • Hatha, yin, and restorative yoga are beneficial for reducing neck pain. Unless you’re experienced, it’s best not to do fast, powerful yoga.
  • Be easy and gentle with yourself. Enjoy the process and the practice, and meet yourself at whichever point you find yourself on a daily basis.
  • Focus on doing at least 10 to 20 minutes of yoga per day, even if it’s only to relax in a few restful positions.
  • Be mindful of your posture throughout the day.

References

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Wells Syndrome – Causes, Symptoms, Treatment

Wells Syndrome or eosinophilic cellulitis is characterized clinically by an acute dermatitis resembling cellulitis and histopathologically by dermal eosinophilic infiltration.

Wells syndrome is a rare eosinophilic disorder that primarily affects the skin. Affected people typically develop a skin rash that is often preceded by itching or burning skin. The rash consists of raised, red, swollen areas that may be warm to the touch.

Wells syndrome is a rare, idiopathic dermatosis with recurrent, erythematous, urticarial plaques that become more indurated and subsequently heal with mild pigmentation. The course of the disease is mild despite occasional constitutional symptoms. Although Wells syndrome is usually sporadic, familial,[] neonatal,[] and childhood[] cases have been reported. There is wide polymorphism in the clinical and histological presentation of the disease, depending upon the nature and location of the infiltrate.[] Blood eosinophilia may or may not be found

Pathophysiology

The pathogenesis of Wells syndrome is obscure. Many triggering factors have been reported including insect bites, viral infections (parvovirus B19, herpes simplex virus, varicella zoster virus, mumps virus), parasitic infections (Ascaris, Toxocara canis, Giardia), bacterial or fungal infections, drugs (antibiotics, non-steroidal anti-inflammatory drugs, thiazide diuretics, anti-TNF, biomedicines) and vaccines. Association of Wells syndrome with other diseases has also been described such as hematologic malignancies (chronic myeloid leukemia, chronic lymphocytic leukemia, polycythemia vera, non-Hodgkin lymphoma), malignant tumors, ulcerative colitis, eosinophilic granulomatosis with polyangiitis (Churg–Strauss syndrome), hypereosinophilic syndrome). Wells syndrome may be prior, revealing, or concomitant to these diseases. The fortuitous nature of some of these situations cannot be ruled out, but one must remain vigilant in case of prolonged evolution beyond 6 months, persistent eosinophilia and/or systemic manifestations associated with Wells syndrome.

The most interesting associations from a pathogenic point of view are certainly those belonging to the spectrum of eosinophilic diseases, such as Shulman syndrome, Churg-Strauss syndrome, and hypereosinophilic syndrome. Wells syndrome could be the first clinical manifestation of these diseases.

The physiopathogenic links between the triggering factors and the associations mentioned above are not clearly established. Inappropriate activation of a Th2-like T lymphocyte clone, synthesizing IL-5, and other eosinophil-stimulating cytokines in response to various, often unidentified, antigenic stimuli is the commonly accepted assumption.

Causes of Wells Syndrome

The pathogenesis of Wells syndrome is unknown. It may be explained as an inappropriate eosinophilic reaction to a wide variety of stimuli due to an abnormal function of eosinophil regulatory systems. Many triggering factors have been proposed: insect bites, drugs, allergic contact dermatitis, an underlying myeloproliferative disorder, and infections (e.g., dermatophytes, viruses, Toxocara canis).

Symptoms of Wells Syndrome

  • Plaque type
  • Annular granuloma-like
  • Urticaria-like
  • Papulovesicular
  • Bullous
  • Papulonodular
  • Fixed drug eruption-like.

Diagnosis of Wells Syndrome

Histopathology

The histological images vary according to the progressive stage of the lesions. Initially, significant edema and a dermal infiltrate of eosinophils are seen. Some of the eosinophils are degranulated. The sub-acute stage is characterized by images called “flame-figures,” located in the mid to deep dermis. The flame-figures are composed of a central part consisting of collagen fibers and eosinophilic granules, surrounded by a histiocytic and eosinophilic infiltrate. Subsequently, eosinophils tend to disappear and are replaced by phagocytic granulomas, consisting of histiocytes and sometimes giant cells, around the flame figures. The absence of vasculitis is an important negative sign.

The flame-figures are not specific to Wells syndrome and can be observed in many other skin conditions in which degranulation of eosinophils occurs including prurigo, eczema, arthropod bite, scabies, bullous pemphigoid, pemphigus vegetans, and eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome).

The study in electron microscopy shows many eosinophils in degranulation, expressing signs of membrane cytolysis. Intact or fragmented free granules are seen in the dermis around the collagen fibers, thus forming the flame figures.

The diversity of clinical aspects of Wells syndrome is probably explained by the level of eosinophilic infiltrate, which may be dermic (superficial or deep), hypodermic thus giving an image of eosinophilic panniculitis, or even subcutaneous.

History and Physical

Wells described the first patient in 1971 as “recurrent granulomatous dermatitis with eosinophilia.” He later renamed the disease “eosinophilic cellulitis.” In 1979, Spiegel and Winkelmann proposed the eponym “Wells syndrome.”

Wells syndrome has a sudden onset. In the classic form, symptomatology is marked by large well limited inflammatory erythematous and edematous patches that are often covered with vesicles or bullae. The lesions are located preferentially on the trunk and the extremities. The eruption is preceded by sensations of itching or burning. General signs are rare. A low fever can be associated with the cutaneous symptoms. The evolution in the following days is marked by an extension of the patches, which take an annular configuration, with the center healing while the border becomes purple. Inflammatory signs regress within approximately 10 days while the plaques become indurated. Restitutio ad integrum usually occurs in 4 to 6 weeks. Recurrence is the rule, with variable locations. The period between recurrences varies from a few months to several years; however, the prognosis remains good with long-term recovery.

Evaluation

Biologically, the main element is peripheral blood eosinophilia which is found in about 50% of cases during the acute phase.

  • Diagnosis is by complete blood count (CBC) – However, in some cases, a more accurate absolute eosinophil count may be needed.[rx][rx]
  • Specific test – for causative conditions are performed, often including chest x-ray, urinalysis, liver and kidney function tests, and serologic tests for parasitic and connective tissue diseases.
  • The stool – is often examined for traces of parasites (i.e. eggs, larvae, etc.) though a negative test does not rule out parasitic infection; for example, trichinosis requires a muscle biopsy.[rx]
  • Elevated serum B12 or low white blood cell  – alkaline phosphatase, or leukocytic abnormalities in a peripheral smear indicates a disorder of myeloproliferation.[rx] In cases of idiopathic eosinophilia, the patient is followed for complications. A brief trial of corticosteroids can be diagnostic for allergic causes, as the eosinophilia should resolve with suppression of the immune over-response.[rx]
  • Marrow aspiration and biopsy – Neoplastic disorders are diagnosed through the usual methods, such as bone marrow aspiration and biopsy for the leukemias, MRI/CT to look for solid tumors, and tests for serum LDH and other tumor markers.[rx]
  • The evaluation for primary eosinophilia – should begin with screening peripheral blood for FIP1L1- PDGFRA gene fusion. Diagnostic testing should start with a peripheral smear. Cytogenetic testing and FISH analysis can be performed on peripheral blood as well.
  • Concurrent cytophilic or cytopenias – if present, can help for diagnosis. In that case, bone marrow biopsy, along with karyotype and genetic screen of chromosomes, may be required.
  • B12 level and tryptase level – along with cytogenetic/immunophenotypic testing and marrow findings, help diagnose chronic mastocytosis, acute/chronic myeloid leukemia, myelodysplastic syndrome, MDS/MPN overlap. When skin rashes are present, skin biopsy helps to diagnose cutaneous disorders like pemphigoid, eczema, mycosis fungoides, Sezary syndrome. Imaging of the chest helps diagnose aspergillosis, Loeffler syndrome, Churg Strauss syndrome. An ultrasound abdomen helps to evaluate for splenomegaly. Stool testing helps to assess for parasitic infections.

Treatment of Wells Syndrome

The treatment of Wells syndrome is not codified. Apart from the etiological treatment of a triggering or associated disease, corticosteroids and dapsone are the two main treatments for Wells syndrome.

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Medical Treatment Physical Modalities
Topical UVB (ultraviolet B)
    Potent corticosteroids PUVA (psoralen plus UVA)
Systemic
    Glucocorticoids
    Antihistamines
    Minocycline
    Griseofulvin
    Dapsone
    Cyclosporine
    Interferon-alpha

 

[/stextbox]

Therapeutic Agents
  • Corticosteroids – It is the reference treatment for all reactional dermatoses whether neutrophilic or eosinophilic. General corticotherapy reduces the duration and importance of relapses in 10% of cases. Initial doses range from 0.5 to 1 mg/kg/day, with rapid tapering. During the decrease of the treatment, it is not uncommon to observe a relapse of the lesions, which can lead, in certain cases, to a real corticosteroid dependence. Local corticosteroids represent an interesting alternative to general corticosteroids, especially in superficial forms, with inconsistent results of the order of 50%. However, it should not be used in deep hypodermic or extended forms.
  • Dapsone – Dapsone is an interesting therapeutic alternative to corticosteroids, with doses ranging from 50 to 200 mg daily. It would reduce the duration of relapses. The optimal duration of treatment is not clearly defined but can be several months in the absence of adverse effects requiring discontinuation of treatment. It can be used alone or in combination with other treatments, including corticosteroids. It can also serve as a relay for general corticosteroids, especially in cases of corticosteroid dependence.
  • Antihistamines – Antihistamines, especially hydroxyzine if pruritus, is important and maybe tried as a first intention, because of their excellent tolerance even if their effectiveness does not seem to exceed 25% of the cases. They sometimes allow the practitioner to avoid the use of general steroid treatment. The dosage varies from 50 to 100 mg/day. Hydroxyzine can be combined with other antihistamines.
  • Methotrexate and other treatments – These are all anecdotal, with their interest being reported in only a few clinical cases. Colchicine, PUVA (psoralen and ultraviolet A) therapy, interferon-alpha, cyclins , synthetic antimalarials, ciclosporin, and anti-TNF agents can be mentioned.
  • Typically treated – with oral or topical corticosteroids such as Prednisone. Other medications that may be used to treat this condition include antifungal drugs, antibiotics, immunosuppressants, and/or antihistamines (H1 receptor antagonists).[rx][rx]
  • Phototherapy – narrow band ultraviolet (UV) B preferred to broadband UVB, as well as psoralen plus UVA (PUVA), is often used as an adjuvant to the above therapeutic modalities.

Therapeutic Strategy

In most cases, general corticosteroids (10 to 80 mg daily) allow rapid healing. Tapering the dose over one month is generally well tolerated. Continued low-dose therapy with corticosteroids allows preventing recurrences. Dapsone may be prescribed as first-line treatment in low inflammatory forms. It also seems to give good results in case of corticosteroids resistance . IFN-alpha and IFN-beta could represent interesting alternatives. For mild cases, topical corticosteroids may be sufficient. Finally, the treatment of an associated disease, when it is found, is essential. It must be prescribed as a first-line treatment and can cure Wells syndrome.

In aggressive forms of the disease, second-line cytotoxic agents and stem cell transplants have proven some benefit. Antibody use against interleukin-5 (IL-5) (mepolizumab), the IL-5 receptor (benralizumab), and CD52 (alemtuzumab), as well as other targets on eosinophils, continues to be an active area of investigation. Timely intervention is vital to reduce morbidity and mortality.

Treatment is directed toward the underlying cause.[rx] However, in primary eosinophilia, or if the eosinophil count must be lowered, corticosteroids such as prednisone may be used. However, immune suppression, the mechanism of action of corticosteroids, can be fatal in patients with parasitosis.[rx]

References

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Grazes/ Scrapes – Causes, Symptoms, Treatment

Grazes/ Scrapes/An abrasion is a partial thickness wound caused by damage to the skin and can be superficial involving only the epidermis to deep, involving the deep dermis. Abrasions usually involve minimal bleeding.[rx] Mild abrasions, also known as grazes or scrapes, do not scar or bleed because the dermis is left intact, but deep abrasions that disrupt the normal dermal structures may lead to the formation of scar tissue. A more traumatic abrasion that removes all layers of skin is called an avulsion.

Types of Abrasions

Abrasions are classified into three types:

  • Linear or scratch abrasions – Linear abrasions are caused by tangential forces resulting in denuding of the epidermis. Linear abrasions are the simplest of injuries and tend to heal by primary intention, without any sequelae. Linear abrasions have significant medicolegal importance, especially when seen over the neck, inner thighs, and genitalia. Linear or semicircular injuries are classically seen as a result of nail scratches, and their presence on the inner aspect of thighs and around female genitalia may indicate resistance in cases of sexual assault. Likewise, nail scratch abrasions on the neck may be suggestive of throttling.
  • Grazed or brush abrasions – Grazed abrasions are usually multiple in presentation and result from friction against a broad, rough surface. These are most commonly seen in cases of road traffic accidents as well as in sports falls. Grazed abrasions are caused by the dragging of the body against a rough surface, resulting in the scrapping of the epidermis. The depth of grazed injuries varies depending on the irregularity of the surface, as well as the speed tugging the body. Extensive, grazed abrasions are sometimes referred to as “brush burns.”
  • Patterned abrasion – Patterned abrasions are a result of perpendicular force on the epidermis, resulting in an impression of the offending item. Patterned abrasions can be further sub-classified as pressure abrasion and impact abrasion, depending on the duration of contact with the offending object.
    • Pressure abrasions result from prolonged compression of the epidermis. The force required to produce pressure abrasion is, however, minimal. E.g., the use of rough material for hanging produces a ligature mark that is an imprint of the material used.
    • Impact abrasions result from a swift blow and require considerable force. Patterned abrasions resulting from blows, collisions, and run-over, etc. are examples of impact abrasions.

By degree

Abrasions on the elbow and lower arm. The elbow wound will produce a permanent scar.
  • A first-degree abrasion involves only epidermal injury.
  • A second-degree abrasion involves the epidermis as well as the dermis and may bleed slightly.
  • A third-degree abrasion involves damage to the subcutaneous layer and the skin and is often called an avulsion.

Pathophysiology

Abrasions range from a break in the epithelial lining to damage to the deeper structures, including nerves, blood vessels, muscles, tendons, organs, and even bone.

Forensic investigations are necessary to determine the extent of the injury and the antemortem or postmortem nature of the wound. The presence of vital reactions, including hemorrhage, cell infiltration, granulation tissue, etc. are indicative of the antemortem quality of an abrasion.

Causes of Grazes/ Scrapes

  • Cuts and scrapes happen when your skin is accidentally broken or worn away. This can be the result of a fall, banging against a hard object, or being cut by something sharp.
  • We all get cuts sometimes, but some people are more prone to these injuries than others. Children, for instance, almost always have some sort of minor skin damage just from playing. Others more likely to get cuts and scrapes include older people and people who have delicate skin because of certain illnesses or medications.

Symptoms of Grazes/ Scrapes

If the wound isn’t healing or you notice any of these signs of infection, call your doctor right away:

  • Redness, swelling, and warmth
  • Increasing pain
  • Pus or drainage from the cut
  • Fever
  • Red streaks around the wound
  • Discharge or pus coming from the cut or scrape
  • Fever
  • Increased pain
  • Foul odour coming from the cut
  • Redness, swelling, or warmth in the affected area

Diagnosis of Grazes/ Scrapes

Hostory and Physical exam

Relevant history should be documented, including the time of injury, cause, and mechanism of injury, as well as other relevant details pertaining to the causation as well as management of the injury.

Abrasions are commonly associated with physical trauma; this could result from falls and impact against hard and uneven surfaces, as well as the pressure of impending objects. They are commonly seen along with other forms of blunt force trauma, such as contusions and lacerations.

While the physical examination of abrasions is important for treatment, the medicolegal examination of abrasions is considerably more significant. Abrasions could be present over any part of the body, frequently seen over the exposed parts of the body, especially the head and neck, as well as the extremities. When found over the neck or genitalia, they tend to have a particular significance that could be precarious, if wrongly interpreted.

The physical examination should include the type, size, shape, color, location, size, depth, association with other injuries as well as the presence of extraneous material. The scientific collection and evaluation of these extraneous materials can provide valuable information regarding the scene of a crime, and in linking the suspect to the crime.

The systematic management of the injury for medical and medicolegal purposes will ensure not just treatment of the injury but also help in providing justice to the victim. The medicolegal examination is especially important in injuries over the head and neck and around the genitalia. Injuries around the genitalia could be vital in identifying sexual violence, while minor injuries over the head and neck could be an indication of more serious underlying injuries.

Evaluation

Abrasions are usually simple in nature and frequently small in size. These abrasions usually heal by the first intention and do not leave any scarring. However, the involvement of large surface area can lead to healing by second intentions, resulting in scar formation. This is particularly seen in individuals susceptible to keloid hyperplasia and should be started on steroid therapy to prevent keloid formation.

The medicolegal investigation may require a biopsy of the abrasion for histological examination. The histological examination investigates the stage of wound healing to provide an estimate of time of injury. Wound healing involves a series of coordinated cellular changes that include bleeding and coagulation, inflammatory response, regeneration, and remodeling. The regeneration process further involves migration and proliferation, while remodeling involves extracellular matrix protein and collagen synthesis, as well as the formation of new parenchymal and connective tissue. These processes are four time-dependent phases:

  • (i) bleeding and coagulation, begins immediately
  • (ii) inflammation, also begins without delay;
  • (iii) regeneration, begins in days and lasts for the primary part of the acute healing phase; and
  • (iv) remodeling, begins weeks after the injury and can last for longer than a year.

The information may be further augmented by histochemical analysis of inflammatory cells and cell mediators. This may assist in informing the law enforcement about the time of occurrence of injury. A skin biopsy is relatively painless and can even be performed in the living if required. Histopathology of an injury can provide vital information for the investigation.

Infection of the injury is another concern and should be managed judiciously. At the same time, the emergence of antibiotic resistance in many organisms should ensure strict follow-up to evaluate compliance and adherence to the full protocol. Injuries should also be swabbed and sent for culture and sensitivity.

Treatment of Grazes/ Scrapes

Abrasions are usually simple, minor injuries that do not require much medical intervention. A sponsored study showed that wet healing, using polyurethane and hydrocolloid plasters, are found to be more efficient and effective in wound healing.

First aid for abrasions

An abrasion means that the surface layers of the skin (epidermis) has been broken. Thin-skinned bony areas (like knees, ankles and elbows) are more prone to abrasions than thicker, more padded areas. The scraped skin of abrasion can contain particles of dirt.

First aid treatment includes:
  • Clean the wound with a non-fiber shedding material or sterile gauze, and use an antiseptic such as Betadine. If there is embedded dirt, Savlon may be used as it contains an antiseptic and a surfactant to help remove debris. Rinse the wound after five minutes with sterile saline or flowing tap water.
  • Don’t scrub at embedded dirt, as this can traumatize the site even more.
  • Cover the cleaned wound with an appropriate non-stick sterile dressing.
  • Change the dressing according to the manufacturer’s instructions (some may be left in place for several days to a week). If you reapply antiseptic, wash it off after five minutes and then redress the wound.

The abrasion should be cleaned and any debris removed. A topical antibiotic (such as neomycin or bacitracin) should be applied to prevent infection and to keep the wound moist.[rx] Dressing the wound is beneficial because it helps keep the wound from drying out, providing a moist environment conducive for healing.[rx] If the abrasion is painful, a topical analgesic (such as lidocaine or benzocaine) can be applied, but for large abrasions. a systemic analgesic may be necessary.[rx] Avoid exposing abraded skin to the sun as permanent hyperpigmentation can develop.

Due to the loss of the epidermis, the outermost layer of protection of the body, abrasions are particularly susceptible to Clostridium tetani and Staphylococcus aureus, particularly in sports injuries. Tetanus toxoid status should always be checked. Abrasions should be cleansed and dressed, protecting the area from reinjury. Debridement may be required, especially if dirt or other contaminants are found embedded. Prevention of infection is the primary objective of any medical intervention. Antibiotic ointments may be applied; after confirming the allergy history, a dressing may be necessary, depending on the area and depth of the injury. Systemic antibiotics may be indicated and should be judiciously prescribed.

Facial abrasions and considered more serious as these have a higher risk of cicatrization and should be cleaned, debrided, and dressed daily. Dressings may require skin adhesives like the combination of gum mastic, styrax, alcohol, and methyl salicylate or tincture of benzoin.

Cuts that require medical attention include the following signs:

  • They are deep (doctors usually are more concerned with how deep a cut is rather than how long it is, because of the concern that deeper tissues like blood vessels, nerves, or tendons may be damaged)
  • They expose any muscle tissue (red) or fat tissue (yellowish)
  • They stay open if you let go of the sides of the cut
  • They are on a joint or in an area where healing might be difficult (stitching might be needed to keep it closed)
  • They remain visibly dirty after being cleaned.
  • They continue to bleed longer than 10 minutes.

If no medical attention is needed, treat the cut in the following way:

  • Cleanse the cut with tap water. You can also use a mild soap. This process helps remove any debris and bacteria from the cut. Avoid using cotton or wool to clean the cut because fibres may get into the wound and cause infection.
  • You can use astringents, alcohol, and antiseptics (e.g., hydrogen peroxide) on the area surrounding the cut, but it is best to avoid using them directly on or in the cut, as they can disrupt the natural healing process.
  • Dress the wound with an over-the-counter topical antibiotic to help prevent infection, if necessary.
  • Cover the wound with a dressing (cloth bandage) that can help keep the cut moist. If you are not sure what type of dressing to use, ask your pharmacist for a recommendation.

Factors influencing wound repair

Traumatized skin heals at different rates, depending on individual and environmental factors, which include:

  • the person’s general state of health
  • age – older skin repairs itself at a slower rate than younger skin
  • the functioning of the immune system
  • dietary factors – skin needs proper nutrition
  • external temperature and weather conditions
  • stress on the injury site, such as friction
  • whether or not the wound becomes infected
  • smoking and some drugs (discuss with doctor or chemist)
  • pre-existing medical conditions, such as some types of vascular disease.

References

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Hirsutoid Papillomas – Causes, Symptoms, Treatment

Hirsutoid Papillomas/Pearly Penile Papules, also known as papillomatosis corona penis, corona capillitii, hirsuties coronae glandis, papillae coronis glands, and hirsutoid papillomas, are benign lesions of the penis.

Pearly penile papules (PPP) are painless and benign lesions that present in rows around the corona of the glans penis in late adolescence or early adulthood. Although asymptomatic, they are often mistaken for sexually transmitted infections such as condyloma acuminata

Hirsuties coronae glandis (also known as hirsutoid papillomas[rx] and pearly penile papulesPPP)[rx] are small protuberances that may form on the ridge of the glans of the human penis. They are a form of acral angiofibromas.[rx] They are a normal anatomical variation in humans and are sometimes described as vestigial remnants of penile spines, sensitive features found in the same location in other primates. In species in which penile spines are expressed, as well as in humans who have them, the spines are thought to contribute to sexual pleasure and quicker orgasms.[rx][rx] It has been theorized that pearly penile papules stimulate the female vagina during sexual intercourse.[rx] In addition, pearly penile papules secrete oil that moistens the glans of the penis.[rx]

Causes of Hirsutoid Papillomas

Pearly penile papules are considered normal anatomical variant.

Penile papules are a normal anatomic variant. They are not due to sexual activity or lack of hygiene. They are not infectious or contagious, unlike genital warts. They are not malignant or pre-malignant.

The exact role of pearly penile papules is not clear to date. They might be vestigial of penile snipes, which are seen in primates and other mammals and may promote sexual pleasure.

Symptoms of Hirsutoid Papillomas

Pearly penile papules do not cause any other symptoms to develop with them.

Once a man has developed pearly penile papules, they typically remain for life. The growths can fade with age, but they do not tend to change shape, color, or spread further over time. Given the similarity in their appearance to some other conditions, such as genital warts, any men that experiencing other symptoms alongside the growths should seek medical attention.

Other conditions that resemble pearly penile papules include:

  • genital warts
  • Fordyce spots
  • molluscum contagious

For example, growths that begin to itch or cause any discomfort may indicate the presence of an underlying condition that a doctor should examine.

Diagnosis of Hirsutoid Papillomas

History and Physical

Although pearly penile papules are usually asymptomatic, they are often mistaken by males who carry them for genital warts, thus causing concerns of having a sexually transmitted infection. This fear of infection may cause tense couple relationships, as it raises questions about fidelity within the couple. One study from Singapore showed that approximately one in seven men, who were examined in a sexually transmitted infection clinic, had only pearly penile papules and no infection.

The level of patient concern appears to be related to the size of the papules. According to a study, two-thirds of males with moderate-to-large pearly penile papules have concerns about their lesions, while one-third of those with less-noticeable papules are worried.

Evaluation

Penile pearly papules are flesh-colored or white, dome-shaped or filiform papules. Their size ranges from 1 mm to 4 mm. Pearly penile papules are arranged in rows around the corona of the glans penis, mainly on its dorsal aspect. There can be one row or many rows. The papules may encircle the entire glans and even have ectopic locations on the penile shaft.

The differential diagnosis includes genital warts, molluscum contagiosum, lichen nitidus, and sebaceous hyperplasia of the penis.

Genital warts are viral tumors induced by human papillomavirus. However, no viral particles are found in pearly penile papules. Nevertheless, the coexistence of both conditions may be observed, and genital warts might be seen in about 1% of males with pearly penile papules.

Molluscum contagiosum may be located on the penis as a sexually transmitted infection. However, these lesions have a larger size and are umbilicated in their center. They rarely have an exclusive location on the corona of the glans.

Lichen nitidus may be limited to the genital area. It manifests as small translucent papules that may involve the glans. However, lichen nitidus lesions are smaller and are usually not limited to the corona of the glans.

Sebaceous hyperplasia is usually seen on the face, but rare cases of the penile location were reported. Lesions usually occur on the ventral aspect of the penile shaft and are white to yellow papules.

In the case of diagnostic difficulty, dermoscopy and histopathology are helpful tools.

On dermoscopic examination, penile pearly papules are arranged according to a grape-like or a cobblestone pattern. They exhibit a white or pink color, with central comma-like, hairpin, or dotted vessels. Such a vascular pattern is not specific, as it may be observed in genital warts. However, pearly penile papules, unlike genital warts, do not show desquamation which manifests as an irregular reflection.

Histopathologically, pearly penile papules share the same features as angiofibroma. Microscopic examination shows an acanthotic epidermis, with elongated rete ridges, overlying dilated vessels located in the papillary dermis. There is usually a dermal proliferation of stellate fibroblasts and marked concentric fibrosis.

Treatment of Hirsutoid Papillomas

Because of the benign nature of pearly penile papules, as well as their possible resolution with age, treatment is not indicated. However, some patients feel distressed or have important cosmetic concerns. Furthermore, about half of males who are reassured of the benign nature of their pearly penile papules want to remove them. Some of them may use inappropriately over-the-counter topicals for common warts, which may cause injuries and scarring.

Pearly penile papules removal is based on physical treatments, namely, cryotherapy and laser therapy.

Studies having assessed cryotherapy are scarce. Two sessions of liquid nitrogen induced good cosmetic results with no pigmentation in a few patients.

  • Ablative lasers and Carbon dioxide laser vaporization – leads to the complete removal of pearly penile papules. This procedure is painful and requires local anesthesia. The vascularization of the penis may cause bleeding during laser sessions but also makes the reepithelialization faster, so that wound healing is achieved within a week. Post-procedure wound management may be inconvenient for patients. Furthermore, there is a risk of scarring and/or pigmentary changes.
  • Unlike continuous-wave and pulsed modes – fractionated carbon dioxide laser causes less tissue damage with fewer adverse effects, but it may require more than one treatment to achieve acceptable cosmetic results.
  • One to six sessions of ablative 2940 nm erbium YAG laser – cleared pearly penile papules in 45 males. Wounds healed within two weeks. No recurrence, no scarring, and no residual pigmentation were noted at the one-year follow-up.
  • Fractional nonablative 1550 nm erbium laser – is less painful than ablative devices and produces only microscopic skin damage which heals rapidly. Up to five treatment sessions were reported to be necessary to obtain a good cosmetic result. Pulsed dye laser may be indicated in pearly penile papules treatment, as it is reported to give good aesthetic results, with a few side effects after one to three sessions.
  • CO2 laser ablation – The high vascularity of penile tissue allows for rapid healing after laser-induced thermal injury, but this also predisposes the patient to bleed during the procedure. The continuous wave mode of the CO2 laser provides better hemostasis and operative field visualization than the short pulse mode [. Using the fractionated CO2 laser, one group reported more than 90% resolution after a single treatment. Another group concluded that two to three treatments were sufficient to completely resolve lesions without adverse effects in both light and dark skin types. While the CO2 laser exposes the underlying tissue, reepithelialization generally occurs within 5 to 7 days. Nevertheless, the procedure requires anesthesia and increases the risk of scarring and infection. Additionally, postoperative management, including home dressing changes, can be inconvenient to patients. The CO2 laser can also lead to postinflammatory pigmentation changes in dark skin types. Despite this fact, two groups reported complete lesion resolution with no adverse pigmentary events.
  • Using the ablative 2940-nm Er – YAG laser, Baumgartner treated 45 patients for one to six sessions. All lesions were successfully cleared with no adverse effects and no recurrence after 1 year. Notably, many of the patients in this study had prior failed treatment attempts with agents such as podophyllin, cryotherapy, and topical fluorouracil plus salicylic acid. Ablated areas healed within 2 weeks after treatment. Despite sustaining up to six laser sessions, no scarring or pigmentation changes were noted.
  • Fractional resurfacing with the 1550-nm erbium laser – has shown complete clearance in one patient after five treatment sessions. Unlike ablative approaches, this laser was relatively painless and did not produce open wounds in the skin. Additionally, there was no lesion recurrence after 1 year

References

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