CT scan appearances in a patient with lipoid proteinosis

Lipoid proteinosis is a rare disorder and is inherited in an autosomal recessive pattern. It was first described in 1929 (Urbach and Wiethe) and since then about 200 cases have been reported, mainly in patients with a known European ancestry. The pathogenesis is unknown; the only constant feature is deposition of an amorphous hyaline material that stains with PAS reagent in the affected tissues. These include in particular the mucous membranes of the mouth and larynx and the skin, though other organs may be involved (Hofer, 1973; Heyl, 1963).