Computed tomography findings in lipoid proteinosis: report of two cases

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Abstract
Lipoid proteinosis (Urbach–Wiethe disease (Urbach, E and Wiethe, C, Lipoidosis cutis mucosae, Virchows Arch. Patholog. Anat., 273, 285–319 (1929)) is a rare generalized disease with autosomal recessive inheritance. It most often involves the skin and mucosal membranes of the aerodigestive tract; but also involves the central nervous system, lung, lymph nodes and striated muscles. We present the computed tomography findings in the cranium and larynx of two siblings with lipoid proteinosis.

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