Otophyma, Rhinophyma and Telangiectatic Rosacea - A Rare Combination in a Female Patient.

BACKGROUND: Rosacea is an inflammatory facial dermatosis seen more frequently in adults in their second half of life. The phymas are a particular subtype with sebaceous gland hyperplasia and progressive fibrosis. CASE REPORT: We report on the rare simultaneous occurrence of telangiectatic rosacea, otophyma and rhinophyma in a 50-year-old female with psoriatic arthritis, chronic lymphedema of the legs, and metabolic syndrome.
CONCLUSION: Despite the preference of rhinophyma and otophyma to the male gender, their occurrence in females needs to be considers in the differential diagnosis of dermatoses of head and neck. Early diagnosis and appropriate medical treatment improve outcome and help to avoid surgery.

A 50-year-old woman with a history of psoriatic arthritis, chronic lymphedema of the legs, and metabolic syndrome presented with a combined facial dermatosis and chronic swelling of the outer ears.

On examination, we observed a central facial persistent erythema with some telangiectasias and chronic lymphedema of the cheeks, and irregular nasal surface with redness and some pustules confirming the diagnosis of rosacea, erythematotelangiectatic type, with mild rhinophyma.

The outer ears were characterized by bilateral swelling involving the ear helix, anthelix and conchal fossa with a partial obstruction of the outer ear’s canal (Fig. 1a-c). Microbial swabs were taken from there which identified Pseudomonas aeruginosa, Turicella otitidis, and Achromobacter. Mycology remained negative. Imaging by thoracic X-ray, abdominal ultrasound and Duplex sonography were unremarkable.

Figure 1

Clinical presentation. (a) Centrofacial erythema with mild rosacea. (b) and (c) Bilateral otophyma with partial obstruction of the outer ear’s canal

The diagnosis of bilateral otophyma with rhinophyma and telangiectatic rosacea was confirmed. We initated systemic drug therapy with minocycline 50 mg twice daily for 6 weeks combined with topical metronidazole ointment for the face and topical fusidinic acid four outer ear’s canals.

There was a stepwise improvement of inflammation and swelling. Treatment was well tolerated.

The phymas are part of the rosacea spectrum characterized by sebaceous hyperplasia, fibrosis and localized lymphedema. The most common type is rhinophyma [1]. Otophyma is a rare subtype which can be uni- or bilateral. It affects men more often than women. The disease results in disfigurement of the outer ears. In very rare cases otophyma can be associated with conductive hearing loss because of the obstruction of the external auditory canal. On clinical examination, edematous swelling with or without erythema and peau d’orange appearance are characteristic while papules and pustules are absent [2-9].

Differential diagnosis includes a variety of skin diseases such as relapsing polychondritis, erysipelas, subcutaneous emphysema, contact dermatitis and urticarial, leprosy and auricular petrosis [1, 7].

Treatment is according to recommendations for rosacea in general with metronidazole, azelaic acid, or ivermectin topically, for the inflammatory rosacea and topical alfa-2-adrenergic inhibitor brimonidine tartrate for erythematous rosacea. Tetracycline, azithromycine or isotretinoin are used orally [1, 10].

For treatment of advanced otophyma debulking surgery in analogy to rhinophyma surgery is an option with excision of the lymphedematous skin and defect closure by free skin transplant. Defect closure can be realized with split-skin or full-skin transplants. Decortication is another surgical option using different ablative techniques such as laser, radiosurgery or dermabrasion followed by healing by second intention [8, 9].

In conclusion, the knowledge of the rare rosacea subtype otophyma is important for dermatologists, ENT, and plastic surgeons.