Basal Cell Nevus Syndrome


MSH: Hereditary disorder consisting of multiple basal cell carcinomas, odontogenic keratocysts, and multiple skeletal defects, e.g., frontal and temporoparietal bossing, bifurcated and splayed ribs, kyphoscoliosis, fusion of vertebrae, and cervicothoracic spina bifida. Genetic transmission is autosomal dominant.,JABL: A syndrome of nevoid basal cell carcinomas, odontogenic keratocysts of the jaws, skeletal anomalies, and intracranial calcifications with more than 100 different signs and symptoms occurring in variable clusters. Basal-cell carcinoma, although the primary characteristic of this syndrome, may not be present in all affected adults. The syndrome occurs in about 1 in 200 patients with basal-cell carcinoma. Mental retardation complicates about 5%. Basal-cell carcinoma associated with coarse sparse hair and milia is referred to as the Bazex syndrome.,NCI: A genetic condition that causes unusual facial features and disorders of the skin, bones, nervous system, eyes, and endocrine glands. People with th

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