Rhinophyma is a disfiguring nasal deformity due to the proliferation of sebaceous glands and underlying connective tissue. The name itself is broken down into “rhis,” derived from Greek meaning nose, and “phyma,” also Greek, for skin tumor. While rhinophyma is the most common subtype of these tumor-like swellings, other sites include the following[rx]:
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Mentophyma (chin)
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Metophyma (forehead)
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Gnatophyma (chin)
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Otophyma (ears)
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Bleharophyma (eyelids)
Acne rosacea is the precursor condition to later development of rhinophyma and was linked to this disease as the ultimate expression of rosacea in 1846 by Ferdinando Hebra Von (1816-1880). [rx] Historically, the condition has been known to exist as seen in a 15th Century painting by Domenico Ghirlandaio (1490), “An Old Man and His Grandson,” which shows a man with a large rhinophyma (See Figure).
This disfiguring disorder is essential to treat, as patients are subject to psychological distress and respiratory issues when alar thickening can obstruct the external nasal valves. An additional social challenge is a commonly presumed link to excessive alcohol use. The direct causal relationship between rhinophyma and alcohol has not been substantiated and has promoted a social stigma, as colloquial names for this condition include “whiskey nose” and “gin blossom.” [rx] [rx]“Potato nose” is another common lay description of this type of nose. Hollywood has used rhinophyma to indicate distasteful or villainous characters: in “Snow White and the Seven Dwarfs” (1937), the villain Queen was given a rhinophyma, besides deep rhytids and periorbital swelling.
Causes of Rhinophyma
Progression through acne rosacea begins with pre-rosacea, vascular rosacea, and inflammatory (acne) rosacea, eventually leading to rhinophyma development. Rosacea often starts between the ages of 20 to 30 with excessive facial flushing exacerbated by vasoactive substances, including but not limited to caffeine and alcohol. Next, the nasal skin thickens and develops consistent erythema due to increased vascularity. Subsequently, patients develop papules and pustules dispersed throughout the face and at times, the scalp and torso: this stage is acne rosacea. A subset of those with acne rosacea then proceeds to rhinophyma development. While the underlying bony structure remains unchanged, the surrounding nasal skin hypertrophies with the preferential expansion of the nasal tip and alae along with potential airway obstruction.[5] Chronic infection often ensues as the sebaceous fluid thickens and traps bacteria. Finally, hyperplasia and fibrosis of the sebaceous glands and connective tissue occur. The skin mite Demodex folliculorum, known to live in pores and hair follicles, has been linked to this condition with unclear timing of a primary or secondary disease manifestation.[rx][rx]
Pathophysiology
Mechanisms of the pathogenesis of rosacea, and in turn rhinophyma, have not been completely elucidated and are thought to be multifactorial involving a combination of neurovascular dysregulation and innate immune responses. Initially, vasodilation leads to fluid leakage into the dermal interstitium and matrix predisposing to inflammation, fibrosis, and characteristic erythematous skin due to telangiectatic growth. Increased mast cell presence has been appreciated in all forms of rosacea and appears in the highest density when papules and pustules begin to develop.
Diagnosis of Rhinophyma
History and Physical
Rhinophyma is diagnosed clinically with erythema, telangiectasias, and thickening of the skin in the nasal region. The lower two-thirds of the nose is affected more than the upper third. There is no specific test for rhinophyma. Diagnostic confirmation is by histology.[1]
Rosacea was divided into four stages by Wilkin in 1994: pre-rosacea, vascular rosacea, inflammatory rosacea, and late rosacea. Rhinophyma belongs to the “late rosacea” stage.[8]
Further refinement of Rosacea in 2002 by the National Rosacea Society (NRS) allowed standardization of rosacea.[9] The diagnosis of rosacea requires the presence of one or more primary features and one or more secondary features.
Primary features
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Transient erythema (blushing)
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Non-transient erythema (persistent redness)
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Papules
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Pustules
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Telangiectasia
Secondary features include:
Evaluation
No laboratory or radiographic tests are necessary for this condition. Clinical evaluation of the patient determines the type and grade of rhinophyma. The severity and sub-types of rhinophyma get classified in different ways.
The first is a division into four clinical variants: glandular, fibrous, fibroangiomatosis, and actinic.
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Glandular rhinophyma: is primarily due to the expansion of sebaceous glands
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Fibrous rhinophyma is due to hyperplasia of connective tissue
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Fibroangiomatous rhinophyma contains fibrosis, telangiectasias, and inflammatory lesions
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Actinic rhinophyma: elastic fibers grow in nodular masses producing disfigurement[10]
An additional severity scale, defined by Freeman et al. in 1970, is also helpful. Beginning with early vascular type, rhinophyma progresses to:
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Moderate diffuse enlargement
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Early localized tumor
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Extensive diffuse enlargement
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Extensive with localized tumor diffuse enlargement[rx]
This condition can also be broken down by a grading system into minor, moderate, or major rhinophyma, used by researcher El-Azhary et al. in 1991.[rx] Under this system, rhinophyma is
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Minor when presenting with telangiectasias accompanied by minor thickening of the skin
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Moderate if skin thickening is accompanied by lobules
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Major is appropriate when prominent nodules and nasal hypertrophy are appreciated[rx]:
The NRS established a standard grading for rosacea and phymatous changes:
Severity level:
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0: Absent
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1: Follicles are patulous but no contour changes
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2: contour change without nodularity
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3: change in contour with the nodular component
Once the rhinophyma has undergone evaluation, a discusison of options is next. Viable choices always include the possibility of not performing any surgical intervention. In the presence of severity levels 1 or 2, the patient may elect to continue to observe. It is wise to follow these patients by taking clinical photographs, including the chin-up position and the lateral views.
Treatment of Rhinophyma
Management of Rosacea
While rosacea responds well to treatment, this does not prevent or treat rhinophyma. Rosacea treatment centers around antibiotics and oral isotretinoin, best utilized in mild to moderate severity. Isotretinoin aids symptom reduction by lowering sebum production and sebaceous gland size. Conversely, topical Retin-A has the potential to worsen rosacea due to enhanced irritation and sensitivity of increasingly erythematous and heavy telangiectatic skin.
Management of Rhinophyma
Patients must be off oral isotretinoin before surgery as it can prevent reepithelialization.[rx] The research proposes that topical metronidazole decreases skin inflammation by inhibiting the generation of reactive oxygen species opposed to reduction of skin bacteria.[rx] While tetracyclines are known for their bacteriostatic effects, it is their anti-inflammatory properties that benefit rosacea patients but does little for rhinophyma.[rx] Historically, X-ray therapy was utilized in the 1920s as it was shown to decrease sebaceous gland size and promoted atrophy of the pilosebaceous unit. However, this treatment is out of favor due to the causation of secondary skin malignancies.
Advanced rhinophyma treatment requires surgical measures to remove tissue. The principle aim of surgery is to reduce the hypertrophied sebaceous glands and recontour the nose. Secondarily, techniques are used to promote re-epithelialization of the nose. As rhinophyma occurs in a noticeable central region of the face, surgery requires careful steps[rx]:
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Reduction of hypertrophied tissue
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Re-contouring of the nasal region
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Controlling excessive bleeding
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Preventing postoperative complications.
Utilizing multiple modalities during surgery is common. Excisional methods carry an additional advantage of providing a specimen for pathologic evaluation, an option not available in ablative techniques due to complete tissue destruction.
Surgery
- Full thickness resection with reconstruction via flap or graft – this approach offers benefits of immediate coverage of the open wound, avoids excessive heat damage, and eliminates recurrence risk by removing all pathologic tissue including underlying cancer. However, this approach has fallen out of favor as flaps and grafts risk failure, grafts can have an incomplete skin color match and also require more operative wounds.
- Partial-thickness tissue removal – this method preserves the pilosebaceous unit. By only excising superficial tissue and retaining the underlying adnexal structures, reepithelialization occurs by secondary intention. It can be done with a standard scalpel and disposable razor blade, making this method cost-effective. Difficulties of this method include the risk of scarring with unintentional removal of the sebaceous unit and achieving hemostasis for a clear visual field. The use of electrocautery is often necessary to control bleeding and add additional nasal refinement.[rx] The use of a heated Shaw scalpel for partial thickness excision can lower the heavy bleeding seen in cold scalpel excision. Operating at 160 to 200 degrees C, it provides bulky tissue reduction and thermal energy for hemostasis. Risks of heated scalpel excision include mild scarring, burns, and potential for slight nasal alar collapse.
- Cryosurgery excisions – this is no longer widely performed. While its benefits entail relatively low intraoperative pain and lack of cartilage damage, this procedure often requires more than one visit with additional risks of scarring, dyschromia, and difficulty in contouring the nose.
- Dermabrasion – dermabrasion is used primarily as an adjuvant treatment post bulk removal to allow for precise nasal contouring.[5]
Ablative treatment
Many different ablative approaches are options in treating rhinophyma:
- Electrosurgery – this approach can both coagulate and cut using a wire loop or epilating needle.
- Electrocautery – heat is utilized from an outside source, while electrosurgery uses radiofrequency electricity to generate heat within the tissue. Both methods have proven beneficial to provide a nearly bloodless cost-effective procedure.[rx] Caution is necessary when employing these methods, especially when cauterizing large blood vessels, as thermal energy can heat surrounding tissue and underlying cartilaginous tissue producing texturization of the skin, high risk of postoperative scarring, and necrosis.
- Coblation – “cold ablation” is an ablative method driven by radiofrequency to excite electrolytes within a conductive medium. Charged ions form a field used to de-epithelialized tissue at low generated temperatures (less than 90 degrees C) decreasing thermal damage risk.[rx]
Laser treatment
Early treatments were with the argon laser: although selective coagulation of capillaries is possible, the depth of destruction of tissue is not predictable.
CO2 laser treatment has the ability to both cut and vaporizes the skin. Utilizing a 10600 nm wavelength, the laser is primarily absorbed by water, allowing for a lower depth of penetration with the ability to reach up to .5mm beyond the visibly burned layer. As the laser is obliterating the sebaceous glands, sebum gets expressed, which serves as a surrogate depth marker as squeezing of the skin during surgery without apparent sebum excretion indicates an appropriate stopping point to avoid scaring. It requires less thermal energy compared to electrocautery and electrosurgery, allows for a bloodless surgical field, necessitates simple post-operative care, and has an overall low scarring risk. The surgeon can utilize CO2 laser along with bulk scalpel removal for more precise contouring. Initial scalpel reduction before using the CO2 laser also allows tissue for histopathologic assessment. Common disadvantages include hypopigmentation, dilated pores due to the destruction of prior sites of follicular cysts and fibrosed sebaceous glands, expensive equipment cost, and lengthy procedure time. Complete reepithelization takes approximately three weeks.[17][18] The Er: YAG laser is an alternative laser option. Operating at the ideal wavelength for water absorption, 2940 nm, it has a smaller heat damage zone of fewer than 50 micrometers allowing for a reduced time to reepithelialization of only 1 to 2 weeks. However, it can only provide minimal hemostasis.[rx]
Other lasers with a successful track record include the diode laser (808 nm), the Nd: YAG laser (1064 nm) and the KTP laser (532 micrometers).
Plasma and Radiofrequency
THe more recently, we have had success using Helium plasma in combination with radiofrequency: this is a unique energy-delivery mode which causes instantaneous heating but also instant cooling in tissues. Whereas bulk heating for more than 152 seconds is needed to a temperature of more than 65 degrees centigrade when using other devices like radiofrequency or ultrasound, helium plasma-RF energy raises tissue temperature to 85 degrees C within 0.044 seconds with the minimal transmission of heat to surrounding tissues; this allows us to ablate the nodules on the nose sequentially with little bleeding. The healing is similar to that seen after the CO2 laser.[rx] Redness and pinkness of the skin can take longer to abate than seen with the CO2 laser.
Non- surgical Treatments undergoing research
Fibrotic cytokines TGFb1 and TGFb2 are proposed to play a role in the skin thickening seen in rhinophyma. Payne et al. have researched the in vitro outcomes of the anti-estrogen medication tamoxifen on fibroblasts with TGFb2 production and secretion in cultured rhinophymatous skin. Results included both decreased fibroblast function and TGFb2 down-regulation. Thus far, this medication has also demonstrated fibrosis inhibition in Dupuytren contracture.[rx]
Complications
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Septal hematoma
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Septal abscess
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Avascular necrosis of nasal septal cartilage leading to saddle deformity
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Nasal obstruction
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Blowout fractures: Extraocular muscle entrapment and diplopia
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Nasolacrimal duct injury: Due to the close relationship of the duct to the nasal bones
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Fracture of cribriform plate and cerebrospinal fluid (CSF) rhinorrhoea
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Inability to reduce: Fractures that cannot be reduced by closed techniques are candidates for open reduction.[rx]
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Airway compromise and hemorrhage.[rx]
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Nasofrontal duct and or lacrimal duct disruption as a result of direct damage or due to displaced fracture segments.[rx]
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Facial deformity, as full correction of telecanthus or nasal depression can be difficult to achieve, and some patients will retain a degree of asymmetry. Depending on the surgical approach, patients may experience temporary or permanent paralysis and or anesthesia of the forehead. Scars that cannot be hidden in the hairy scalp or skin folds may be prominent.[rx]
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Mental health, as patients with facial injuries are at greater risk of developing post-traumatic stress disorder or anxiety-related disorders. Particularly those who were victims of assault.[rx]