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American Journal of Roentgenology, Vol 99, 817-825, Copyright © 1967 by American Roentgen Ray Society

BASAL CELL NEVUS SYNDROME: ITS ROENTGENOLOGIC SIGNIFICANCE

REVIEW OF THE LITERATURE AND REPORT OF FOUR CASES

MELVIN H. BECKER M.D.1, ALFRED W. KOPF M.D.2, and ADAM LANDE M.D.3

1 From the Department of Radiology, New York University School of Medicine
2 From the Department of Dermatology, New York University School of Medicine
3 From the Section of Radiology, Cumberland Hospital

The basal cell nevus syndrome is a heredi tary disorder, involving ectodermal and mesodermal structures, which results in a number of clinically and roentgenographically demonstrable abnormalities.

The main features of this syndrome are multiple basal cell epitheliomas of the skin, cysts of the jaws, and various skeletal anomalies.

Hyporesponsiveness to the phosphaturic effect of parathyroid extract, ectopic deposits of calcium, and shortened fourth metacarpals suggest a relationship of the basal cell nevus syndrome to hypoparathyroidism and/or gonadal dysgenesis.

The frequency of medulloblastomas (4 in about 150 reported cases) deserves further investigation.


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Copyright © 1967 by the American Roentgen Ray Society.