A case report of Lipoid Proteinosis

Abstract

Objective: Lipoid proteinosis is a rare, autosomal recessive disease, characterized by persistent non-inflammatory popular lesions in the skin and mucous membranes. Papules are deposits of hyaline materials, similar to basement membrane substances in different connective tissues. The pathogenesis of disease is still unknown. Treatment is nonspecific and symptomatic. There are few reports of good results after treatment with dimethylsulphoxide and etretinate. Case: This is a case report of a nine-year old girl with typical popular lesions on free margins of the eyelids erosive, crusted and scarring skin lesions and hoarseness were also noted. After treatment with etretinate for 6 months, skin biopsy examination confirmed the diagnosis exosire and crusted, hyperkeratotic lesions disappeared. There were fewer papular lesions on the eyelids that were not so thick as before and hoarseness alleviated.

Keywords

• lipoid proteinosis
• mucous membranes
• skin lesions