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A case of oral recurrent ulcerative lesions in a patient with lipoid proteinosis (Urbach-Wiethe disease).

Research paper by Sergio S Sargenti Neto, Jonas Dantas JD Batista, Antônio Francisco AF Durighetto

Indexed on: 17 Nov '09Published on: 17 Nov '09Published in: British Journal of Oral and Maxillofacial Surgery



Abstract

Lipoid proteinosis is a rare autosomal recessive genodermatosis characterized by deposition of amorphous hyaline material in different parts of the body, especially the skin, mucous membranes of the upper aerodigestive tract, and internal organs. A clinical manifestation of LP usually begins as a hoarseness and failure cry soon after birth or in the first years of life. However, other conditions may occasionally appear few years later. Oral cavity is most extensively affected area by the disease and the main oral abnormalities include diffusive infiltration of white pea-size plaques and stiffening of the tongue as well as inability to protrude it. In this report, we describe the case of a woman affected by LP presenting recurrent ulcerative lesions in mouth associated with xerostomia.