A Case of Nevoid Basal Cell Carcinoma Syndrome / 대한피부과학회지

ABSTRACT

Nevoid basal cell carcinoma syndrome (basal cell nevus syndrome) is characterized by multiple basal cell carcinomas, cysts of jaw and several other congenital anomalies on skeletal, neurologic and reproductive system. It is inherited as an autosomal dominant trait. We found a 12-year-old girl with multiple basal cell carcinomas, bifid ribs, palmar pits, ophthalmic hypertelorism, ectopic calcification of falx cerebri and medulloblastoma and diagnosed it as nevoid basal cell carcinoma syndrome. Her 2-year-old younger sister showed no specific abnormality. We dessicated the basal cell carcinomas and nevi which were suspected to perform malignant transformation with carbon dioxide laser, and are to continue the follow-up study and treatment for patient and her family members.