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Anais Brasileiros de Dermatologia
On-line version ISSN 1806-4841
LIMA, Letícia Rathlew de et al. Lipoid proteinosis: a report of two cases. An. Bras. Dermatol. [online]. 2003, vol.78, n.6, pp.723-727. ISSN 1806-4841. http://dx.doi.org/10.1590/S0365-05962003000600008.
Lipoid proteinosis (Urbach-Wiethe disease) is a rare disorder with autosomal recessive inheritance, characterized by progressive deposition of hyaline substance in the skin, mucous membranes and internal organs. Clinically, there are papular and nodular lesions on the face, with areas of infiltration in the elbows, knees and hands; the tongue is firm and hoarseness may be present at birth. We describe two cases of this disease in the same family (brother and sister).
Keywords : lipoidproteinosis.