Pediatric chondrodermatitis nodularis helicis (CNH) in a child with Beckwith‐Wiedemann syndrome (BWS)

Abstract

Chondrodermatitis nodularis helicis is an idiopathic degenerative process that presents as a painful nodule, papule, or ulcer on the helix or antihelix. It predominantly affects adults and is thought to be associated with trauma to the ear. We describe a case of pediatric chondrodermatitis nodularis helicis occurring in a child with a history of Beckwith-Wiedemann syndrome that was successfully treated with an excisional biopsy and relief from a recurrent source of pressure on the ear. | 389Pediatric Dermatology BRIEF REPORT to be associated with trauma to the ear, most commonly from pressure while sleeping, though reports exist of CNH triggered by hats, helmets, telephones, and headphones. 1 Vascular insufficiency, underlying connective tissue disease, thermal, and actinic damage have also been proposed to contribute to CNH. 2 Chondrodermatitis nodularis helicis is predominantly a disease of adults, with only 7 reported cases in the pediatric literature (Table 1). Three of the seven documented cases of pediatric CNH occurred in children with underlying connective tissue disease, and researchers have proposed this as a predisposing factor for the development of CNH in these cases. Researchers have also proposed that genetic and developmental abnormalities of the ear may contribute to the development of CNH. 3,4 We expect that the imbalance of genes controlling growth on chromosome 11p15 contributes to the development of CNH, although the details of how these genes affect chondrodermatitis development is still unclear. Common BWS findings of ear pitting or ear creases were absent in this patient. However, as this child had larger-than-average ears and left-sided lateralized overgrowth, we hypothesize that persistent pressure from headphones on his enlarged ear predisposed him to developing CNH. An extensive clinical differential diagnosis for CNH includes calcinosis cutis, gout tophi, reactive perforating collagenosis, verruca vulgaris, basal cell carcinoma, squamous cell carcinoma, actinic keratosis, and weathering nodule of the ear. Although CNH in a pediatric patient is rare, it should be considered in any young patient presenting with a tender papule or nodule on the external ear. When CNH is suspected, providers should try to identify any contributing factors, with the understanding that their absence does not preclude the diagnosis.Surgical removal is the first line treatment for suspected CNH, as it allows histopathologic assessment of the lesion in question and has a higher cure rate than common nonsurgical techniques like pressure relief or topical nitroglycerine. 6,7 The efficacy and safety of other nonsurgical techniques like topical or intralesional steroids, collagen injection, cryotherapy, laser therapy, platelet-rich plasma, topical bacitracin, and photodynamic therapy are less well studied. 7

Painful symmetric ear papules in a 16‐year‐old girl

“…Prior literature sites eight published cases of pediatric CDNH, sometimes in association with connective tissue diseases. 3 There is a report of a 12-year-old boy with Beckwith-Wiedemann syndrome who developed unilateral CDNH of the left ear due to wearing over-the-ear headphones for school activities nine hours a day. 3 Another case describes a 15-year-old boy who developed unilateral chondrodermatitis nodularis of the right antihelix from wearing over-the-ear headphones with video gaming.…”

“…3 There is a report of a 12-year-old boy with Beckwith-Wiedemann syndrome who developed unilateral CDNH of the left ear due to wearing over-the-ear headphones for school activities nine hours a day. 3 Another case describes a 15-year-old boy who developed unilateral chondrodermatitis nodularis of the right antihelix from wearing over-the-ear headphones with video gaming. 4 To our knowledge, this is the first case describing pediatric bilateral CDNH occurring from ear-bud use.…”