Basal cell nevus syndrome: its roentgenologic significance. Review of the literature and report of four cases.

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.