Chondrodermatitis nodularis helicis (CNH) is a chronic painful or crusted, 4- to 6-mm, solitary nodule, primarily on the upper part of the ear (most commonly on the right side). The presence of pain, which increases the likelihood that a person will seek treatment, clinically distinguishes CNH from other cutaneous tumors in the differential diagnosis that produce painless ulceration.
It is roughly 5 times more prevalent in males (72.9%),1 with an average age of onset of 65 years.2 However, CNH has been reported in females3 and rarely in individuals younger than 20 years. According to a PubMed search of articles indexed for MEDLINE and a Google Scholar search using the terms chrondodermatitis nodularis helices child, only 6 cases of CNH have been reported in the pediatric population.4-8 The youngest reported case was a 9-month-old infant.8 Including the present case, males and females in the pediatric population are equally affected; 4 patients had an underlying dermatomyositis,7 rheumatoid nodule,8 or systemic disease, including systemic lupus erythematosus and Beckwith-Wiedemann syndrome.5,9 Chronic intermittent pressure from headwear was the etiologic agent in the remaining cases.4 Recognizing that CNH can occur in young patients and can be associated with underlying autoimmune disease helps direct management and avoid overly invasive treatment.
Case Report
A 17-year-old adolescent boy presented with a painful ulcerated papule on the right upper helix of 3 months’ duration (Figure 1). The patient habitually slept on the right side, pressed a cell phone to that ear, and wore a tight-fitting visor while lifeguarding, which, along with solar damage, all may have contributed to the disease process. He was otherwise in good health, without a history of underlying systemic disease. Given the patient’s extensive occupational sun exposure, biopsy of the lesion was taken under the impression of CNH vs squamous cell carcinoma or basal cell carcinoma.
Figure 1. Chondrodermatitis nodularis helicis. An erythematous 7-mm papule with central ulceration on the right upper helix of 3 months’ duration.
Histopathologic analysis revealed a central area of ulceration with edematous degenerated dermal collagen and overlying inflammatory crust, characteristic of CNH (Figure 2A). Biopsy in this patient demonstrated classic histopathologic findings of CNH, including a central area of epidermal ulceration capped by an inflammatory crust and an underlying edematous degenerated dermal collagen (Figure 2B).
Figure 2. A, Histopathology revealed variable epidermal acanthosis at the margins of the central defect, which in rare cases assumes the proportions of pseudoepitheliomatous hyperplasia (H&E, original magnification ×10). Palisaded histiocytes surrounded the necrobiotic zone. B, Irregular slit-like spaces extended into degenerated dermal collagen (H&E, original magnification ×40).
Following biopsy, the patient was advised of this diagnosis and recommended to avoid applying pressure to the area with cell phones or hats or when sleeping to prevent recurrence. At 3-month follow-up, no residual lesion remained.
Comment
Pathogenesis
The exact cause of CNH is unknown but is probably the result of prolonged and excessive pressure on the ear that leads to ischemic injury to cartilage and skin. The external location of CNH, lack of bony support, and exquisitely thin padding or insulation in the form of subcutaneous tissue make the small dermal blood vessels supplying the outer ear vulnerable to compression. Dermal inflammation; edema; and necrosis from trauma, cold, or actinic damage also can help initiate CNH. This disruption of blood perfusion to the external ear also inhibits the ear’s ability to heal. A cycle of pressure from objects such as a pillow or cell phone, followed by inadequate healing, leads to secondary perichondritis and remodeling of perichondrial arterioles, which is demonstrated histologically by the presence of perichondrial fibrous thickening, mild chronic inflammation, collagen degeneration, hyalinization, and rarely necrosis or calcification. Healed lesions often show dermal fibrosis overlying perichondrium.