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Chondrodermatitis Nodularis Helicis

Key points

  • Chondrodermatitis nodularis helicis (CNH) is an uncommon, strikingly painful lesion on the helix or antihelix of the ear, which often appears as a crusted ulceration mimicking a cutaneous malignancy.
  • Diagnosis may be made by clinical exam and the symptom of pain, but biopsy of commonly performed to exclude cutaneous malignancies
  • Treatment is aimed at protecting the area with local measures, with a number of topical, intralesional, and ultimately surgical treatment approaches available

Introduction

Chondrodermatitis nodularis helicis (CNH) (or chondrodermatitis nodularis chronica helicis) is a painful lesion on the outer ear that is markedly more common in men than women, with a ratio approaching 10:1. CNH tends to occur in older patients, typically in patients in their 50s, 60s, or 70s. While the overall incidence is unknown, CNH is likely more common than recognized in the literature, due to underreporting. CNH occurs as a very painful ulceration, which may be crusted, arising almost invariably on the helix or antihelix of the ear. Some CNH lesions may appear dome shaped or have a raised rim, which may appear inflamed and erythematous. Typical lesions are small, ranging from 3 to 10mm in size. The vast majority appear as ulcerations, frequently with a thick serous crust. CNH is almost always unilateral, and patients often note the lesions are present on the side of their head that they sleep on. Due to their clinical appearance, CNH lesions are often mistaken for non-melanoma skin cancers (either basal cell or squamous cell carcinomas). Unlike those cutaneous malignancies, CNH is a benign entity. Pain is a critical feature, as CNH is invariably symptomatic, though patients may note a variety of types of pain arising from their lesion.

The differential diagnosis is primarily non-melanoma skin cancers (basal and squamous cell carcinoma). Other entities to consider include solar (actinic) keratoses, cutaneous horns, verruca vulgaris (warts), gouty tophi, or other cutaneous malignancies.

A small skin biopsy is sometimes performed to confirm the diagnosis and exclude those common cutaneous malignancies. Histologically, CNH is defined by increased vasculature, often seen at the edges of the ulcer, with variable amounts of mixed inflammatory cells. Cartilage, when present, may appear degenerated.

The precise cause of CNH is unknown; most feel that the lesion results from repetitive pressure and trauma on an area of photo-damaged skin. While the etiology of CNH is unknown, many have speculated that this may be a form of localized tissue ischemia, either due to local vascular factors (arteriole narrowing), external pressure or trauma, or cold-induced vasospasm. Most patients to not recall a specific inciting trauma, but external factors likely play a role in the disease. It is suspected that minor trauma, such as sustained localized pressure from pillows, telephones, headsets, or other activities of daily living may be sufficient to incite CNH.

Treatment is generally conservative, at least initially. Some patients will experience spontaneous improvement, however, it is more common that patients who seek physicians for this condition will experience persistent lesions if untreated, and most patients experience recurrences.

Initial therapy includes advising patients to avoid pressure to the area. Patients should use a soft pillow, including potentially a pillow with a gap for the ear or a pressure-offloading “doughnut.” They should avoid using telephones on that side and avoid headphones. There is limited data for a variety of local approaches, including light cryotherapy, topical corticosteroids, intralesional corticosteroids, or intralesional collagen/dermal fillers. Recently, topical nitroglycerin has been demonstrated to help some cases of CNH in a small study. Surgical options have long been the standard for recalcitrant cases. Certain lasers, typically ablative lasers, have been used. Curettage and electrodestruction has also been reported to help. In recurrent, refractory cases, surgical excision of the lesion and associated sub-ulcer cartilage is often very effective.