Fifth Phacomatosis

A syndrome of nevoid basal cell carcinomas, odontogenic keratocysts of the jaws, skeletal anomalies, and intracranial calcifications with more than 100 different signs and symptoms occurring in variable clusters. Basal-cell carcinoma, although the primary characteristic of this syndrome, may not be present in all affected adults. The syndrome occurs in about 1 in 200 patients with basal-cell carcinoma. Mental retardation complicates about 5%. Basal-cell carcinoma associated with coarse sparse hair and milia is referred to as the Bazex syndrome.