A case report of Lipoid Proteinosis

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...

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Autor principal: S Tirgar Tabari
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Lenguaje:EN
FA
Publicado: Babol University of Medical Sciences 1999
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Acceso en línea:https://doaj.org/article/4a8296f9c0a9494d8b363ba8f9b15b9b
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spelling oai:doaj.org-article:4a8296f9c0a9494d8b363ba8f9b15b9b2021-11-10T09:24:32ZA case report of Lipoid Proteinosis1561-41072251-7170https://doaj.org/article/4a8296f9c0a9494d8b363ba8f9b15b9b1999-10-01T00:00:00Zhttp://jbums.org/article-1-2943-en.htmlhttps://doaj.org/toc/1561-4107https://doaj.org/toc/2251-7170Objective: 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.S Tirgar TabariBabol University of Medical Sciencesarticlelipoid proteinosismucous membranesskin lesionsMedicineRMedicine (General)R5-920ENFAMajallah-i Dānishgāh-i ̒Ulūm-i Pizishkī-i Bābul, Vol 1, Iss 4, Pp 53-56 (1999)
institution DOAJ
collection DOAJ
language EN
FA
topic lipoid proteinosis
mucous membranes
skin lesions
Medicine
R
Medicine (General)
R5-920
spellingShingle lipoid proteinosis
mucous membranes
skin lesions
Medicine
R
Medicine (General)
R5-920
S Tirgar Tabari
A case report of Lipoid Proteinosis
description 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.
format article
author S Tirgar Tabari
author_facet S Tirgar Tabari
author_sort S Tirgar Tabari
title A case report of Lipoid Proteinosis
title_short A case report of Lipoid Proteinosis
title_full A case report of Lipoid Proteinosis
title_fullStr A case report of Lipoid Proteinosis
title_full_unstemmed A case report of Lipoid Proteinosis
title_sort case report of lipoid proteinosis
publisher Babol University of Medical Sciences
publishDate 1999
url https://doaj.org/article/4a8296f9c0a9494d8b363ba8f9b15b9b
work_keys_str_mv AT stirgartabari acasereportoflipoidproteinosis
AT stirgartabari casereportoflipoidproteinosis
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