Basal cell carcinoma syndrome: report of 10 cases.

Ten cases of basal cell carcinoma syndrome are presented. The jaw cysts were proved histopathologically to be odontogenic keratocysts. The multiple naevoid lesions on the skin were identified as basal cell carcinoma in two cases. Skeletal anomalies and intracranial calcification were present in all cases. A positive family history with others affected by the syndrome was traced in two cases.