Keywords: lipoidproteinosis, skin diseases

Abstract: The authors presente a review of the literature and a report of a Lipoid proteinoses case or Hyalinosis cutis et mucosae or Urbach-Weithe disease. This is a rare disease, that occurs by the deposit of hialin material of unknown bioquimic nature, in the mucose, skin, upper air-digestive way, central nervous system, and in others tissues. The clinicals and otolaryngologicals manifestations are described as well as the complementary tests and clinical evaluation. Because of the rareness and gravity of this disease that does not tend to retroage spontaneously or with treatment, carrying to death, the ENT specialist must previously recognize this disease.