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Basal-Cell Carcinoma Syndrome

Pierre E. Ferrier, MD; Ward L. Hinrichs, MD
Am J Dis Child. 1967;113(5):538-545. doi:10.1001/archpedi.1967.02090200070005.
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IN RECENT years Gorlin and his associates1-3 extensively studied a clinicopathological entity which they called the "multiple basal-cell nevi syndrome." This disorder affects several structures or systems other than the skin, but attempting to find a better name for it, reflecting all its many aspects, would probably be impractical. Therefore, the name basal-cell nevi or nevoid basal-cell carcinoma syndrome has generally been adopted.4 In general, the syndrome consists of: multiple basal-cell carcinomas, often nevoid, multiple mandibular and maxillary epithelial cysts, multiple skeletal anomalies and a typical craniofacial appearance, and endocrine defects and tumors. The syndrome is inherited as an autosomal dominant trait. The present report concerns a family in which it appeared that the disease manifested itself in four generations.

Report of the Family  The pedigree of the family is shown in Fig 1. The propositus (IV-4), a 15-year-old boy, was seen by us because of tall stature


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