UPDATE ON INHERITED BULLOUS DERMATOSES - 08/09/11
Résumé |
The term epidermolysis bullosa, coined by Koebner in 1886,43 refers to a group of inherited bullous disorders characterized by blister formation in response to mechanical trauma. Early on, Hallopeau28 was able to recognize two major categories of bullous disease, simplex (epidermolytic) and dystrophic (scarring). Herlitz described epidermolysis bullosa letalis,32 which was later shown to be part of the third major category of epidermolysis bullosa, the junctional form. The studies of Pearson and collaborators,58 who applied the techniques of electron microscopy to epidermolysis bullosa, helped to establish this technique as a gold standard in distinguishing the three major epidermolysis bullosa subtypes.
Over the past decade, protein and genetic abnormalities have been identified with most types of epidermolysis bullosa. These studies have permitted a better understanding of the molecular pathophysiology of epidermolysis bullosa. While epidermolysis bullosa classification to date has leaned heavily on morphology, this new advance in knowledge permits a biological classification of epidermolysis bullosa that will provide a more rational approach to diagnosis and to future specific therapies. Much understanding has been gained regarding the underlying mechanisms of this group of diseases. To appreciate these new advances and their clinical applications, one needs to understand the basic molecular organization of the dermal-epidermal basement membrane, depicted in Figure 1.
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Vol 17 - N° 3
P. 473-485 - juillet 1999 Retour au numéro
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