Basal cell nevus syndrome의 10년간의 추적연구

Basal cell nevus syndrome-10 years follow up

The basal cell nevus syndrome is a hereditary disease with multiple defects involved organs and systems of both ectodermal and mesodermal origin. Anomalies found in the basal cell nevus syndrome is characterized by the five major category : cutaneous anomalies, dental & osseous anomlies, ophthalmologic anomalies, neurologic anomalies, sexual anomalies. In our cases, we found the multiple jaw cyst, palmar & plantar pits, hypertelorism, epidermal inclusion cyst, sebaceous cyst, congenital missing & transposition of teeth, mental retardation, hypodevelopment of sexual organ in a series of 10 patients visited to Dept. of Oral and Maxillofacial Surgery, School of Dentistry, Kyung Hee University. We have continued to investigate and look for extension their pedigree.