Otolaryngologic findings of five lipoid proteinosis cases

Abstract

Lipoid proteinosis (Urbach-Wiethe disease) is a rare autosomally recessive inherited disease, which presents with various symptoms due to deposits of hyaline material in tissues. Lesions of larynx and skin are frequent. Otolaryngologic findings of 5 cases of lipoid proteinosis were analyzed, and literature was reviewed. We observed involvement of larynx in all cases with skin lesions in our series. The disease was determined in oral cavities of 3 cases, and tongue base of a case.

Keywords

• lipoid proteinosis
• urbach-wiethe disease
• otolaryngology