A Case of Basal Cell Nevus Syndrome with Symmetric Basal Cell Carcinomas on Neck

Basal cell nevus syndrome is an autosomal dominant disorder characterized by developmental and skeletal anomalies, palmo-plantar pits, odontogenic keratocysts, ectopic calcification, and occurrence of various types of tumors including basal cell carcinoma. Within our country, 9 cases at dentistry and 3 cases at dermatology were reported. The patient was a 66-year-old woman. Chief complaint was multiple dark brownish colored papules which were symmetrically distributed around the neck. Excision and biopsy were done under general anesthesia and the wound was healed without complication. Histological examination of the lesion revealed 'basal cell carcinoma'. During the evaluation of metastasis of basal cell carcinoma, at PNS CT we found odontogenic keratocyst on the left alveolar process of maxilla by accident. Physical examination revealed pits of the palm and sole. These are consistent with the major diagnostic criteria for basal cell nevus syndrome. Hereby we report one case of basal cell nevus syndrome.

A Case of Nevoid Basal Cell Carcinoma Syndrome with Multiple, Symmetrically Distributed Basal Cell Carcinomas / 대한피부과학회지

Nevoid basal cell carcinoma syndrome is a rare inherited disorder characterized by multiple cutaneous basal cell carcinoma, pits of the palms and soles, cysts of the jaws, and ectopic calcification of cranial membranes. The patient was a 66 year old female. She complained of multiple, symmetrically distributed black-colored papules on the both sides of the neck. On physical examination, multiple pits were detected in the palms and soles. The histopathologic findings of the papules showed variable shaped and sized tumor nests composed of basaloma cells. On PNS CT for search of metastasis of the cancer, odontogenic keratocyst was detected at the Lt. alveolar process of maxilla by accident. We diagnosed her nevoid basal cell carcinoma syndrome and the lesions on both sides of the neck were removed completely.