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The Korean Journal of Pathology 1995;29(2): 263-267.
Nevoid Basal Cell Carcinoma Syndrome: Report of a case.
Hae Ryoun Park, Young Im Han, Mee Young Sol, Sun Kyung Lee
1Department of Oral Pathology, College of Dentistry, Pusan National University, Pusan, Korea.
2Department of Pathology, College of Medicine, Pusan National University, Pusan, Korea.
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.
Key Words: Nevoid basal cell carcinoma syndrome