Author/Authors :
Shome, K. Kolkata Medical College - Department of Dermatology, Venereology and Leprology, India , Das, I. Burdwan Medical College and Hospital - Department of Dermatology, Venereology and Leprology, India , Saha, A. Burdwan Medical College and Hospital - Department of Dermatology, Venereology and Leprology, India , Jain, N. Burdwan Medical College and Hospital - Department of Dermatology, Venereology and Leprology, India , Seth, J. Burdwan Medical College and Hospital - Department of Dermatology, Venereology and Leprology, India , Rajesh Burdwan Medical College and Hospital - Department of Dermatology, Venereology and Leprology, India
Abstract :
Lipoid proteinosis is a rare autosomal recessive disorder with variable phenotype, caused by defect in extracellular matrix protein-1 and is characterized by deposition of periodic acid- Schiff-positive, diastase resistant material in skin, mucous membrane and internal organs. There are only few reports regarding lipoid proteinosis in literature and in this part of the world. Here, we report a case of lipoid proteinosis in a 29-year-old male with positive family history and widespread distribution involving skin and internal organs. Histopathological finding was consistent with clinical diagnosis of lipoid proteinosis.
