Chondrodermatitis nodularis chronica helicis.

Chondrodermatitis nodularis chronica helicis is a small, painful, scaly nodule of the helix of the ear. It was first described by Winkler in 1915, and it is also known as Winkler k disease. (1) The lesion usually occurs in men older than 50 years; the male-to-female ratio is 10:1. (1) We describe 2 cases.

Case reports

Patient 1. In 2004, a 62-year-old woman presented with a 3-month history of a right ulcerative auricular lesion. She complained of extreme focal pain and bleeding, and she reported that the lesion had progressed rapidly. Her history was significant for a salvage laryngectomy in 2000.

On physical examination, the lesion measured 4 cm and involved the right helix (figure 1). Because suspicion was high for a second head and neck primary, an incisional biopsy was performed. Microscopic analysis revealed marked dermal inflammation with granulation tissue extending down to uninvolved cartilage. The epidermis was hyperkeratotic with foci of psoriasiform hyperplasia and extensive ulceration with scaly crust formation. No dysplasia or malignancy was identified. These findings were consistent with chondrodermatitis chronica nodularis helicis.

[FIGURE 1 OMITTED]

The patient completed 2 cycles of high-dose oral steroids, and the progression of the disease ceased and all symptoms resolved.

Patient 2. In 2003, an 81-year-old Russian-speaking woman presented with right ear pain. Findings on the head and neck examination were significant for an ulcerative lesion that involved the middle of the helical rim (figure 2). Because the patient had a history of skin malignancies, we performed a wide local excision of the lesion. Analysis of the excised specimen detected epidermal ulceration with foci of necrosis in the hyaline cartilage, focal granulation tissue, and epidermal hyperplasia and hyperkeratosis; these findings were consistent with chondrodermatitis nodularis chronica helicis.

[FIGURE 2 OMITTED]

Postoperatively, the patient experienced a complete resolution of all symptoms, and she has had no recurrence of disease.

Discussion

Based on physical examination findings, the differential diagnosis of chondrodermatitis chronica nodularis helicis includes basal cell carcinoma, squamous cell carcinoma, keratoacanthoma, and polychondritis secondary to an autoimmune disease. Therefore, a biopsy of the lesion must be performed to rule out malignancy. Biopsy results positive for Winkler's disease include epithelial hyperplasia, collagen degeneration, focal fibrinoid necrosis, and inflammatory cells with or without cartilage degeneration.

Once malignancy is ruled out and a histologic diagnosis of chondrodermatitis nodularis chronica helicis is reached, the patient can be treated surgically or medically. Moncrieff and Sassoon recommend conservative management, based on their findings that 87% of medically managed cases had resolved by the 1-month follow-up. (2) They also reported that 34% of patients who were treated surgically experienced a recurrence.

References

(1.) Oelzner S, Eisner R Bilateral chondrodermatitis nodularis chronica helices on the free border of the helix in a woman. J Am Acad Dermatol 2003;49:720-2.

(2.) Moncrieff M, Sassoon EM. Effective treatment of chondrodermatitis nodularis chronica helicis using a conservative approach. Br J Dermatol 2004;150:892-4.

From the Head and Neck Cancer Center, Cedars-Sinai Medical Center, Los Angeles.