COMPUTED-TOMOGRAPHY FINDINGS IN LIPOID PROTEINOSIS - REPORT OF 2 CASES

Lipoid proteinosis (Urbach-Wiethe disease (Urbach, E and Wiethe, C, Li poidosis cutis mucosae, Virchows Arch. Patholog. Anat., 273, 285-319 ( 1929)) is a rare generalized disease with autosomal recessive inherita nce. It most often involves the skin and mucosal membranes of the aero digestive tract; but also involves the central nervous system, lung, l ymph nodes and striated muscles. We present the computed tomography fi ndings in the cranium and larynx of two siblings with lipoid proteinos is.