A case of nevus comedonicus syndrome associated with neurologic and skeletal abnormalities

A 12-year-old male was referred to us with recurrent pus discharge from tender nodules on the right axilla dating from the neonatal period. The nodules were black, characterized by scarring with dilated follicular openings and there were black papules filled with comedo-like keratin plugs in both axillae. Physical examination revealed a bowing deformity of the right third finger and retardation in language ability. The patient was referred to the Departments of Neurology and Orthopedics in Chungnam National University Hospital, Korea. Histologic examination of one of the black comedo-like lesions showed a bulbous and dilated infundibulum that contained laminated keratin, indicating a diagnosis of nevus comedonicus. A CT scan of the brain revealed dysgenesis of the corpus callosum, The IQ (intelligence quotient) score of the patient, measured by the Korean Wechsler Intelligence Scale for Child ren-Revised, was 94. The only difficulty noted for ordinary life was learning language. A radiograph of the right hand revealed hyperextension and an ulnar drift deformity of the right middle finger. Corrective osteotomy with external fixation and an iliac bone autograft were performed. Intermittent neurologic follow-up visits were ordered for the noted language deficit. At present the patient only exhibits difficulty in calculation. Oral antibiotics were administered to the skin lesions on occasion for secondary infections and inflammation of the cysts and comedones. Extraction of the comedones was performed as needed.