Six cases of basal cell nevus syndrome

Basal cell nevus syndrome is primarily considered to be an autosomal dominant hereditary disease frequently accompanying basal cell epitheliomas, multiple jaw cysts, anomalies of the rib, palmar and plantar pits, calcification of the cerebro-falx, and various skin and skeletal anomalies.We recently experienced 6 cases of this syndrome. Among them, there were 2 cases of family involvement, a case of a father and his daugther and a case of a mother and her son. In one case, ulcerous nevie was observed in the arm, which was revealed to be a basal cell epithelioma histopathologically after removal of the ulcer. All cases had multiple jaw cysts and they were all of odontogenic keratocysts pathologically. In two cases the jaw cyst recurred.In those patients with basal cell epithelioma and multiple jaw cysts, it se ms necessary to observe for a long follow-up period.