Lipoid Proteinosis of Urbach and Wiethe


Hyalinosis Cutis et Mucosae

Lipoid Proteinosis, Urbach-Wiethe



Urbach Wiethe Disease

Urbach Wiethe Lipoid Proteinosis

Urbach-Wiethe Disease

Urbach-Wiethe Lipoid Proteinosis

Urbach-Wiethe Syndrome

An autosomal recessive disorder characterized by glassy degenerative thickening (hyalinosis) of SKIN; MUCOSA; and certain VISCERA. This disorder is caused by mutation in the extracellular matrix protein 1 gene (ECM1). Clinical features include hoarseness and skin eruption due to widespread deposition of HYALIN.