1

Case report

A 50-year–old woman presented with swelling of the left pinna for the last 15 years in our outpatient department. The swelling was initially the size of a pea, when she received a series of corticosteroid injections, and was considered as keloid by the general physician. There was no history of trauma ear, cough or expectoration, dryness eyes, or similar swelling elsewhere in the past. The swelling was painless, slowly progressive, and not associated with any bleeding, pus, or fluid discharge. She had a history of another course of 10 steroid injections locally 5 years back, advised by skin specialist, but with no relief.

On examination, the left pinna showed a 6 × 4-cm, irregular, firm, normothermic, painless swelling with overlying skin having many puncta and many blackheads ( Fig. 1 ). The skin at places was adherent to underlying tissues. The swelling mainly involved superior portion of the helix, part of triangular fossa laterally, and the adjacent skin of medial and lateral surfaces of the upper one third of the pinna. The swelling was cystic anteriorly (1 × 1 cm), with fluctuation being present. No telangiectasia, papules, and pustules were seen on the swelling or the face; but the patient described flushing after tea consumption or on taking spicy foods. The result of the ophthalmic examination was normal.

Irregularly swollen, bulky ear with many follicular plugs.

All results of routine blood and urine investigations were within normal limits. The patient was advised biopsy, but she demanded complete excision in single sitting. The patient was taken up for complete excision of the swelling along with the cyst under local anesthesia. The lateral inferiorly based flap was elevated after incision along the helix up to the lower margin of swelling. Part of the skin attached to the tissue along with that medial to pinna was removed along with the swelling. The upper part of the posterior margin of the cartilage was bared in the process. The skin flap was reposited, and primary closure was done in single layer. Intra- and postoperative period was uneventful.

The histopathologic examination of the specimen revealed hypertrophy of sebaceous glands, dilated follicular plugs, minimal fibrosis, moderate edema, and a perivascular and perifollicular infiltrate of lymphohistiocytes and plasma cells. No Demodex mites were seen in the follicles. The patient is under follow-up and has no recurrence for the last 10 months.

2

Discussion

Phyma , derived from the Greek word meaning growth , is a disfiguring disorder of the face and is the end stage of rosacea . It is caused by sebaceous gland hyperplasia and hypertrophy and surrounding fibrous tissue proliferation. The phymas have been named according to the site of occurrence : rhinophyma , commonly seen on the nose; gnatophyma , occurs on the chin; metophyma , for forehead; otophyma , when one or both ears are involved; and blepharophyma, when seen on eyelids. Otophyma or otic phyma (rosaceous lymphedema of the ear) has been rarely reported and can present as either unilateral or bilateral ear involvement with or without coexisting facial rosacea. To our knowledge, this is only the second case report of otophyma in the otolaryngology literature but the third in the English-language literature .

Rosacea is a common chronic cutaneous disorder of unknown etiology. It is manifested by transient or persistent facial erythema, telangiectasia, edema, papules, and pustules most frequently localized to central face. Although rosacea is common in females, otophyma and other phymas are rare. Ocular changes such as blepharitis and conjunctivitis are seen commonly . Patients may point out certain exacerbating factors, especially for flushing, such as heat or sunlight exposure, alcohol or hot beverage intake, stress, menstruation, and sometimes certain foods . Rosacea is classified into 4 subtypes: erythematotelangiectatic, papulopustular, phymatous, and ocular . The severity of each subtype is graded as 1 (mild), 2 (moderate), or 3 (severe) . This classification is useful in guiding the therapy.

In this article, we wish to limit the discussion to the phymas. Rhinophyma is the most frequent phymatous manifestation. It is also known as whisky nose , rum blossom , hammer nose , elephantiasis nose , or potato nose . The etiology, pathology, and the treatment of rhinophyma as mentioned in the literature apply for otophyma as well. They occur more commonly in white men aged 40 to 60 years. It is interesting to see that the disease acne rosacea is 3 times more common in females but its end stage, the phyma, occurs more commonly in males, with ratio of 20:1 . It begins as normal flush reaction in adolescence; but with time, the blood vessels of the nose become dilated, the skin thickens at the tip, and sebaceous glands hypertrophy. As the deformity worsens, cysts, pits, nodules, fissures, and lobulations cover the tip and alar portion of the nose. The skin gets irregularly thickened; and follicles are prominent, with foul-smelling inspissated sebum.

The diagnosis of otophyma is made on clinical basis; but sometimes, biopsy is necessary to differentiate from lupus pernio (sarcoidosis of the nose), carcinomas (basal cell , squamous cell , and sebaceous), angiosarcoma , and nasal lymphoma . Not only can additional pathology hide within the phymatous nose, but cancer and other disorders can also actually mimic the disease. The histopathology of phymas reveals hyperplasia and hypertrophy of the sebaceous glands, the duct being elongated, dilated, and plugged. There is irregular fibrous tissue proliferation with inflammatory cell infiltrate and bacteria. Foreign body reactions can be seen, as the Demodex folliculorum mite regularly takes up residence in the pilosebaceous units.

Early lesions of rosacea (erythematotelangiectatic) usually respond to local dermatologic treatment. In late presentations (papulopustular), systemic medical therapy is often required. These include oral antibiotics such as metronidazole for acute exacerbation and tetracycline for long-term management. No antibiotic or retinoid has been shown conclusively to halt the progression from rosacea to phymas or cause regression of existing phyma. Thus, despite the advances in medical therapy for the treatment of rosacea, it has limited success for phymas.

Once phymatous lesion occurs, the deformity only responds to surgical treatment. A wide range of surgical approaches to phymas has been described, all of which usually involve shaving off the hypertrophied tissue with or without immediate resurfacing. These are the following:

• 1)
  

  Full-thickness excision followed by split-thickness skin graft application. The disadvantages are poor color matching and cosmetically poor margins.

  
  
• 2)
  

  Full-thickness excision followed by a full-thickness skin graft. It provides better texture and color; but if residual dermal appendages are left under the graft, recurrent cyst and sinus tract may form. The full-thickness skin can also be harvested locally in the manner of local skin flaps for coverage, as has been done in our case.

  
  
• 3)
  

  Decortication, that is, partial-thickness excision. In this, the phymatous tissue is shaved off in layers up to 2 to 3 mm above the underlying cartilages. The reepithelization is relied on remaining fundi of the hypertrophic sebaceous glands, thus giving normal texture and color match. If one stops resecting above this plane, the tissue remains bulbous; and if overresected, the area will remain raw, granulating, and nonepithelized. This decortication can be performed using cryosurgical techniques, chemical peels, dermabrasion, the Shaw knife (a thermally heated scalpel), and CO ₂ laser. The scalpel and dermabrader often lead to significant bleeding that can compromise accuracy. Thus, most surgeons recommend use of CO ₂ laser, as it allows operation in a near-bloodless field . Postoperative care entails cleansing the wound with hydrogen peroxide twice daily, followed by the application of antibiotic ointment until complete epithelization occurs.

In conclusion, rosacea should be considered as a differential diagnosis in cases with long-standing swelling of the ear. In the current case, anti-inflammatory treatment with intralesional corticosteroids minimally reduced the swelling and other signs of rosacea but did not prevent the progression of lymphedema. Thus, in cases not amenable to medical therapy, surgical approach directed at removal of excess tissue can improve local symptoms and overall cosmetic appearance.