AHMAD FATHIZADEH, M.D.; DAVID F. FRETZIN, M.D.; MURRAY J. FAVUS, M.D.; FREDERIC L. COE, M.D.
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To the editor: In the basal cell nevus syndrome, an autosomal dominant trait with high penetrance and variable expressivity, numerous basal cell epitheliomas appear during the second and third decades accompanied by keratocysts of the jaw, bifid ribs, brachymetacarpalism, frontal and temporoparietal bossing, hypertelorism, and areas of ectopic calcification (1, 2). Other features include palmar and plantar pits, hirsutism, epidermal cysts, oculoneurologic abnormalities, gastric hamartomatous polyps, mesenteric cysts, and ovarian teratoma. The syndrome has been associated with uterine and ovarian fibroma, cystadenoma and theca cell tumors, meduloblastoma, multiple meningiomas, astrocytomas, ameloblastomas, craniopharyngioma, and fibrosarcoma of the jaw. We report here
FATHIZADEH A, FRETZIN DF, FAVUS MJ, et al. Primary Hyperparathyroidism and Basal Cell Nevus Syndrome. Ann Intern Med. 1980;93:147. doi: https://doi.org/10.7326/0003-4819-93-1-147_1
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Published: Ann Intern Med. 1980;93(1_Part_1):147.
Endocrine and Metabolism, Hematology/Oncology, Parathyroid Disorders.
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