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Vesiculobullous disease 

Vesiculobullous disease

Chapter:
Vesiculobullous disease
Author(s):

Fenella Wojnarowska

DOI:
10.1093/med/9780199204854.003.2304

November 28, 2013: This chapter has been re-evaluated and remains up-to-date. No changes have been necessary.

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date: 22 August 2017

The autoimmune blistering diseases have a dramatic clinical presentation, and are significant diseases with substantial morbidity and mortality.

The diseases can be split broadly pathologically into intraepidermal (pemphigus) and subepidermal (pemphigoid, dermatitis herpetiformis and others) groups, the former being characterized by pathogenic autoantibodies to desmosome components, and the latter by pathogenic antibodies to proteins of the basement membrane zone adhesion complex that link the epithelium/epidermis to the underlying mesenchyme/dermis (or genetic mutations of the same proteins). There are concomitant differences in clinical presentation, e.g. blistering lesions present in the subepidermal bullous diseases tend to be less easily ruptured than those observed in intraepidermal bullous diseases....

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