Lipoid Proteinosis / 대한피부과학회지

ABSTRACT

Lipoid proteinosis is a rare autosomal recessively inherited disorder that is characterized by the deposition of hyaline-like material in the skin, oral cavity, and other organs. Microscopically, there is extensive deposition of amorphous eosinophilic material surrounding capillaries, sweat glands and in papillary dermis. Although the pathogenesis of this disease is not well understood, it is believed that it may result from the defect of collagen metabolism leading to abnormal accumulation of noncollagenous glycoprotein. We report a case of lipoid proteinosis in a 20-year-old female that demonstrates the characteristic clinical, histopathological, and ultramicroscopic features of this disease.