Overview of Heriditary Epidermolysis Bullosa

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Review Paper 01/06/2019
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Overview of Heriditary Epidermolysis Bullosa

Saima Anwer Zaman, Saliha Samiullah
Int. J. Biosci.14( 6), 241-249, June 2019.
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Abstract

Epidermolysis is a monogenic disease, constitute several genodermatosis due to metamorphosis in numerous structural and functional cohesion in skin. Epidermolysis Bullosa (EB) is considered a multi-system disorder associated with significant outer-layer manifestation with severe complications in other epithilized organ possibly provided. After 19th century several proceeding in classification has been switched. This review will recapitulate the most recent classification with epidemiological and clinical features; recent effective gene therapy treatment with positive potential focused on EB recently identified forms in autosomal recessive manner one of Junctional Epidermolysis Bullosa (JEB) and other two in EBS (DST-e, EXPH5 and ITGA3).Level of skin cleavage is identified by IFM Immunoflourescence mapping and Electron microscopy EM. Requires far-reaching patient treatment management on advance level because EB addicts a long-term cure and medicines which is an expensive and conviction to stress on financial ground, much more research need to be developed and used in clinical practices. This review provides practical knowledge of disease including classification and phenotypes.

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