Nevoid Basal Cell Carcinoma Syndrome: Report of a case.
- Author:
Hae Ryoun PARK
;
Young Im HAN
;
Mee Young SOL
;
Sun Kyung LEE
- Publication Type:Case Report
- Keywords:
Nevoid basal cell carcinoma syndrome
- MeSH:
Child;
Male;
Female;
Humans
- From:Korean Journal of Pathology
1995;29(2):263-267
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Nevoid basal cell carcinoma syndrome (NBCC syndrome) is a very rare autosomal dominant dermatopathy characterized by a primary triad with variable secondary anomalies. The chief features include nevoid basal cell carcinomas, one or more skeletal anomalies, and multiple odontogenic keratocysts of the jaw. We report a case of NBCC syndrome in a 43-year old male who had multiple nevoid basal cell carcinomas on the retroauricular area, face, chest wall, and back, which have been present since childhood. Skull x-rays revealed relatively well-circumscribed cystic, radiolucent lesions on bilateral rami of both upper and lower jaws, calcification of the falx cerebri, agenesis of the right coronoid process, and a bifid chin. The cystic lesions were histologically confirmed as keratocysts.
