Lipoid proteinosis (Urbach-Wiethe disease): A case report from India | Ear, Nose & Throat Journal Skip to content Skip to navigation

Lipoid proteinosis (Urbach-Wiethe disease): A case report from India

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September 1, 2008
by Kadambari Batra, DLO, DNB, Anil Safaya, MS, and Kiran Aggarwal, MS


Lipoid proteinosis (Urbach-Wiethe disease) is a rare autosomal-recessive anomaly that primarily affects the skin and the mucosa of the upper aerodigestive tract in children. It is caused by hyaline deposits in tissues. Hoarseness secondary to laryngeal involvement is frequently the first presenting feature. It is important to consider this disease in the differential diagnosis of hoarseness because it might lead to life-threatening airway compromise. We report a case of lipoid proteinosis in a 12-year-old girl who presented with hoarseness and skin lesions.

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