J Korean Child Neurol Soc.
2010 May;18(1):147-152.
A Case of Basal Cell Nevus Syndrome
- Affiliations
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- 1Department 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.
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