2011
DOI: 10.1177/0194599811415823a58
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Lipoid Proteinosis: Case Report of Otolaryngological Findings

Abstract: Objective: Lipoid proteinosis, or Urbach-Wiethe disease, was first described in 1929. It is a rare recessive genetic disorder that causes a buildup of hyaline material in the skin and mucosa. The objective of this paper is to report a case of lipoid proteinosis, or Urbach Wiethe disease, emphasizing its otolaryngological manifestations. Method: A 38-year-old patient presented since young childhood with skin lesions on buttocks, face, arms, and legs and a weak cry. Currently, the patient has hoarseness that ke… Show more

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“…Corticosteroids were used in four patients. In one patient, there was no improvement (25%), 17 in two patients, there was a beneficial effect on oral lesions, hoarseness, and skin lesions (50%), 22,23 and in the remaining patient, a positive effect concerning the voice was noted, but this immediately worsened again after the treatment was stopped (25%). 24…”
Section: Corticosteroidsmentioning
confidence: 96%
“…Corticosteroids were used in four patients. In one patient, there was no improvement (25%), 17 in two patients, there was a beneficial effect on oral lesions, hoarseness, and skin lesions (50%), 22,23 and in the remaining patient, a positive effect concerning the voice was noted, but this immediately worsened again after the treatment was stopped (25%). 24…”
Section: Corticosteroidsmentioning
confidence: 96%