Lipoid proteinosis

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Lipoid proteinosis

(Science: disease) a familial disease occurring in the course of latent diabetes, marked by yellowish nodules due to deposits of a protein-lipid complex on the oral tongue and sublingual and faucial areas, translucent keratotic papillomatous eyelid lesions, keratotic lesions on the extremities, and hoarseness.

It is due to a disturbance of lipid metabolism with autosomal recessive inheritance and is frequently associated with intracranial calcifications.

inheritance: autosomal recessive.

Synonym: hyalinasis cutis et mucosae, lipoidosis cutis et mucosae, Urbach-Wiethe disease.