Korean J Pathol.
1995 Apr;29(2):263-267.
Nevoid Basal Cell Carcinoma Syndrome: Report of a case
- Affiliations
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- 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.
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