Necrotic Lesion of the Ear

The Diagnosis: Chondrodermatitis Nodularis Chronica Helicis
Histopathologic examination revealed focal epidermal erosion and ulceration directly overlying the hyaline cartilage with degenerative changes (Figure). The dermis was relatively noninflamed with fibroplasia of the vasculature. The blood vessels indirectly beneath the ulceration were found to be unremarkable with no indications of fibrinoid necrosis, vasculitis, or the presence of thrombi. The patient was informed of the diagnosis, at which point she reported that she slept on the right side. The excisional biopsy site healed well without recurrence of chondrodermatitis nodularis chronica helicis (CNH).

Excisional biopsy specimen of the necrotic area of the right helix (A and B)(H&E, original magnifications ×20 and ×100). Sections demonstrated focal epidermal erosion and ulceration directly overlying hyalinized cartilage with degenerative changes. The dermis was relatively noninflamed with angiofibroplasia. The blood vessels not directly beneath the ulceration were unremarkable.

Chondrodermatitis nodularis chronica helicis, also known as clavus helicis, is a benign, usually solitary, painful lesion. Historically, it was first described in 1915 by Winkler¹ and in the 1960s the most common documented cases were attributed to the headpieces of telephone operators and nuns.² In the early 2000s, cell phones were determined to be a growing cause.³ Chondrodermatitis nodularis chronica helicis is most commonly found on the helix with the antihelix being affected less often.⁴ The condition is more common in men, with a male to female ratio being reported as high as 10:1. Possible causes of this disorder stem from damage to cartilage associated with pressure, sun exposure, cold temperatures, and microvascular disease. Additionally, some researchers have hypothesized that the cartilaginous damage resulting from solar elastosis and minor trauma leaves a susceptibility to CNH. This disorder usually presents as a small, exquisitely tender nodule that may ulcerate and crust.⁴ Chondrodermatitis nodularis chronica helicis may be mistaken for basal cell carcinoma, squamous cell carcinoma, actinic keratosis, and weathering nodules, though CNH tends to be more painful.

The diagnosis of CNH often is clinical but may require a skin biopsy. Histopathology of CNH shows a benign inflammatory lesion with an acanthotic hyperkeratotic epidermis that may be ulcerated. A primarily lymphocytic infiltrate usually is observed with variable presence of histiocytes and neutrophils. Cartilaginous changes range from simple perichondral thickening to notable areas of degeneration with calcification and ossification.⁴

Although the diagnosis of CNH often is straightforward, the remarkable necrosis present in our case made for an interesting differential diagnosis. Pernio, cryoglobulinemia, and levamisole-induced vasculopathy were all considered. Pernio, caused by cold-induced vasoconstriction and hypoxemia, classically presents as erythematous lesions with a symmetrical distribution on acral sites.⁵ Cryoglobulinemia involves proteins that precipitate at cold temperatures causing damage via an occlusive vasculopathy or an immune complex-mediated vasculitis. The presence of cryoglobulinemia is strongly associated with concomitant hepatitis C virus infection.⁶ Ulcerated and purpuric lesions of cryoglobulinemia may become necrotic. Levamisole is a veterinary antihelminthic drug and common cocaine contaminant, often added to cocaine as a cutting agent. Levamisole-induced vasculopathy favors acral sites and often is noted on the ears as purpuric patches, sometimes with necrosis.⁷

Several therapies for CNH have been reported with variable effectiveness.⁸ First-line treatments are the use of pressure-relieving devices including a doughnut-shaped pillow during sleep and intralesional corticosteroids.⁹ Surgical treatments including cryotherapy, simple excision, electrodesiccation and curettage, wedge resection with helical rim advancement flap, punch and graft technique, and CO2 laser have been tried.⁸ Photodynamic therapy and topical nitroglycerine also have shown to be of benefit.^8,9

Our case of CNH is unique because of the remarkable degree of necrosis present on clinical examination. Chondrodermatitis nodularis chronica helicis with such an impressive necrotic presentation is rare. We speculate that the patient's underlying hypercoagulable state may have contributed to the dramatic presentation. It is important to keep CNH in mind when evaluating any necrotic lesion on the ear.