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May 1967

Basal-Cell Carcinoma Syndrome

Author Affiliations

From the Joan Judson Research Unit, Children's Orthopedic Hospital and Medical Center and the Division of Endocrinology and Metabolism, Department of Pediatrics, University of Washington, Seattle.

Am J Dis Child. 1967;113(5):538-545. doi:10.1001/archpedi.1967.02090200070005

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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