Epidermolysis bullosa, junctional

Definition:

Inherited epidermolysis bullosa is a diverse group of disorders characterized by mechanically fragile skin that readily blister. The junctional forms of epidermolysis bullosa are characterized by blister formation within the lamina lucida of the dermal-epidermal basement membrane. Herlitz subtype, the classic form of the disease, shows a severe phenotype that may lead to death during infancy or early childhood owing to infection.

References:

[1]. Daisuke Sawamura, et al. Overview of epidermolysis bullosa. J Dermatol. 2010 Mar;37(3):214-9. [Content Brief]

[2]. Jo-David Fine, et al. Inherited epidermolysis bullosa. Orphanet J Rare Dis. 2010 May 28;5:12. [Content Brief]

[3]. Jo-David Fine, et al. Inherited epidermolysis bullosa: past, present, and future. Ann N Y Acad Sci. 2010 Apr;1194:213-22. [Content Brief]

[4]. Jo-David Fine, et al. Inherited epidermolysis bullosa: recent basic and clinical advances. Curr Opin Pediatr. 2010 Aug;22(4):453-8. [Content Brief]

[5]. Jouni Uitto, et al. Progress in epidermolysis bullosa: from eponyms to molecular genetic classification. Clin Dermatol. 2005 Jan-Feb;23(1):33-40. [Content Brief]

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