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Autoimmun Rev. 2014 Apr-May;13(4-5):477-81. doi: 10.1016/j.autrev.2014.01.011. Epub 2014 Jan 11.

Diagnosis and classification of pemphigus and bullous pemphigoid.

Author information

1
Department of Dermatology, Rabin Medical Center, Beilinson Hospital, Petah Tikva, Israel.
2
Department of Dermatology, Rabin Medical Center, Beilinson Hospital, Petah Tikva, Israel; Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
3
Department of Dermatology, Rabin Medical Center, Beilinson Hospital, Petah Tikva, Israel; Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel. Electronic address: mimouni@post.tau.ac.il.

Abstract

Pemphigus and bullous pemphigoid represent the two major groups of autoimmune blistering diseases. Pemphigus has three major variants: pemphigus vulgaris, pemphigus foliaceus and paraneoplastic pemphigus and is characterized by autoantibodies directed against the cell surface of keratinocytes, producing acantholysis that in turn leads to intraepithelial blisters in the skin and/or mucous membranes. In bullous pemphigoid, the autoantibodies are present at the dermo-epidermal junction and attack the hemidesmosomes, causing subepidermal blister formation. The classification of the major variants of both the pemphigus group and bullous pemphigoid can be based on the combination of clinical, histopathological and immunopathological criteria. Many tools are available for the diagnosis of these entities including biopsy, direct and indirect immunofluorescence, immunoprecipitation, immunoblotting and ELISA. However, currently there are no generally accepted criteria for the diagnosis of these disorders. The present review provides a proposal for diagnostic criteria.

KEYWORDS:

Bullous pemphigoid; Clinical classification; Criteria; Diagnosis; Pemphigus

PMID:
24424192
DOI:
10.1016/j.autrev.2014.01.011
[Indexed for MEDLINE]

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