A Case of Basal Cell Nevus Syndrome.

Son,  Young Jun; Jo,  Han Gil; Kim,  Young Ok; Woo,  Young Jong

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Case Report

Journal of the Korean Child Neurology Society 2010;18(1):147-152.
Published online May 30, 2010.

A Case of Basal Cell Nevus Syndrome.

Young Jun Son, Han Gil Jo, Young Ok Kim, Young Jong Woo

Department of pediatrics, Chonnam National University, Medical School, Gwangju, Korea. yjwoo@chonnam.ac.kr

Abstract

Basal cell nevus syndrome(BCNS) is a rare autosomal dominant disorder characterized by variable developmental anomalies and predisposition to cancers. The main manifestations include multiple basal cell carcinomas of skins, odontogenic keratocysts, facial dysmorphism, skeletal abnormalities including scoliosis and bifid ribs, palmar and plantar pits, calcification of the falx cerebri, and biparietal frontal bossing. We experienced a case of 12-year-old girl with the clinical features of basal cell nevus syndrome.

Key Words: Basal cell nevus syndrome, Odontogenic cysts

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