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Giornale Italiano di Dermatologia e Venereologia 2006 December;141(6):495-505

Copyright © 2006 EDIZIONI MINERVA MEDICA

language: English

Bullous pemphigoid, an autoimmune blistering disease with protean clinical manifestations. Review of recent advances from the bench to the bedside

Lebeau S., Clivaz L., Fontao L., Borradori L.

Department of Dermatology University Hospital of Geneva Geneva, Switzerland


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Bullous pemphigoid (BP) is the most frequent autoimmune subepidermal blistering disease and affects predominantly the elderly. It is associated with autoantibodies directed against the BP antigen 180 (BP180 or BPAG2) and the BP antigen 230 (BP230 or BPAG1), two hemidesmosomal components that promote dermo-epidermal cohesion. The clinical features of BP are extremely protean. While its diagnosis is easily considered in the presence of a pruritic eruption with widespread blistering, atypical variants or early stages of BP with either localized or generalized, excoriated, eczematous, vesicular or urticated lesions may constitute a clinical pitfall. In these cases, diagnosis of BP uniquely relies on the findings of the immunofluorescence microscopy studies as well as on the characterization of targeted antigens. Recently, in addition to the identification of clinical criteria useful for the diagnosis of BP and further improvement of the standard immunopathological investigations, enzyme-linked immunosorbent assays (ELISA) utilizing recombinant proteins of BP180 and BP230 have been developed, that allow the rapid characterization of circulating anti-BP180 and anti-BP230 antibodies with high specificity and sensitivity. These tests represent useful diagnostic tools, but their value for the management and follow-up of affected patients remains to be determined.

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