Lipoid proteinosis in two Indian brothers - a case report from South Bengal
JPAD-Journal of Pakistan Association of Dermatologists. 2014; 24 (1): 89-92
in English
| IMEMR
| ID: emr-157649
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
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Index:
IMEMR (Eastern Mediterranean)
Main subject:
Periodic Acid-Schiff Reaction
/
Skin
/
Basement Membrane
/
Extracellular Matrix Proteins
/
Genes, Recessive
/
Lipoid Proteinosis of Urbach and Wiethe
Type of study:
Case report
Limits:
Humans
/
Male
Language:
English
Journal:
J. Pak. Assoc. Dermatol.
Year:
2014
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