Overview of epidermolysis bullosa
Article first published online: 26 FEB 2010
© 2010 Japanese Dermatological Association
The Journal of Dermatology
Special Issue: Blistering Diseases (pages 193-239)
Volume 37, Issue 3, pages 214–219, March 2010
How to Cite
SAWAMURA, D., NAKANO, H. and MATSUZAKI, Y. (2010), Overview of epidermolysis bullosa. The Journal of Dermatology, 37: 214–219. doi: 10.1111/j.1346-8138.2009.00800.x
- Issue published online: 26 FEB 2010
- Article first published online: 26 FEB 2010
- Received 22 October 2009; accepted 1 November 2009.
- basement membrane;
- bone marrow transplantation;
- 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 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.