Overview of epidermolysis bullosa

Epidermolysis bullosa (EB) is classified into major types – EB simplex (EBS), junctional EB (JEB) and dystrophic EB (DEB) – on the basis of the level of tissue separation within the cutaneous basement membrane zone. Recent advances in research on EB have led to the identification of 10 genes respons...

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Bibliographic Details
Published in:Journal of dermatology 2010-03, Vol.37 (3), p.214-219
Main Authors: SAWAMURA, Daisuke, NAKANO, Hajime, MATSUZAKI, Yasushi
Format: Article
Language:English
Subjects:
basement membrane
Basement Membrane - pathology
blister
Blister - genetics
Blister - pathology
bone marrow transplantation
diagnosis
epidermolysis bullosa
Epidermolysis Bullosa - classification
Epidermolysis Bullosa - genetics
Epidermolysis Bullosa - therapy
Genetic Therapy
Humans
Membrane Proteins - genetics
Neoplasm Proteins - genetics
Plectin - genetics
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Description
Summary:Epidermolysis bullosa (EB) is classified into major types – EB simplex (EBS), junctional EB (JEB) and dystrophic EB (DEB) – on the basis of the level of tissue separation within the cutaneous basement membrane zone. Recent advances in research on EB have led to the identification of 10 genes responsible for EB. The Japanese Ministry of Health, Labor and Welfare has designated JEB and DEB, but not EBS, as specified diseases. However, EBS has a lethal variant and should also be registered as a specified disease. In the Third Consensus Meeting on the Diagnosis and Classification of EB held in Vienna in 2007, it was recommended that Kindler syndrome should be classified as a subtype of EB. Corrective gene therapy is the most ideal therapy for EB, but much more research is required before it can be developed and used in clinical practice. Cell‐based therapies using fibroblasts and bone marrow cells have recently attracted considerable attention.
ISSN:0385-2407
1346-8138
DOI:10.1111/j.1346-8138.2009.00800.x