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Lipoid proteinosis
K Konstantinov1, P Kabakchiev, T Karchev
1Department of Autoimmune Serology, Statens Seruminstitut, Copenhagen, Denmark.
Journal of the American Academy of Dermatology
|August 1, 1992
Abstract:
Lipoid proteinosis (Urbach-Wiethe disease) is a rare, recessively inherited disorder that is characterized by the deposition of hyaline-like material in the skin, oral cavity, and other tissues. It usually appears in infancy with hoarseness. We report a case of lipoid proteinosis in a 10-year-old boy that demonstrates the characteristic clinical, histologic, and ultramicroscopic features of this disease.