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Direct and indirect immunofluorescence staining patterns in the diagnosis of paraneoplastic pemphigus
Author(s) -
Poot A.M.,
Siland J.,
Jonkman M.F.,
Pas H.H.,
Diercks G.F.H.
Publication year - 2016
Publication title -
british journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.304
H-Index - 179
eISSN - 1365-2133
pISSN - 0007-0963
DOI - 10.1111/bjd.14282
Subject(s) - paraneoplastic pemphigus , pemphigus , direct fluorescent antibody , staining , medicine , pathology , immunofluorescence , dermatology , indirect immunofluorescence , antibody , autoantibody , immunology
Paraneoplastic pemphigus (PNP) is a rare but severe multiorgan autoimmune disease that occurs in the presence of an underlying neoplasm. It is clinically characterized by a painful oral stomatitis, and a range of skin eruptions. In PNP, autoantibodies are directed against the intracellular desmosomal and hemidesmosomal plaque proteins of the plakin family: envoplakin, periplakin, desmoplakin, BP230 and plectin. In addition, the desmosomal proteins desmoglein (Dsg) 3 and less frequently Dsg 1 are targeted, as well as the protease inhibitor alpha-2-macroglobulin-like antigen-1 (A2ML1).(1-4) Diagnosing PNP largely depends on the demonstration of these autoantibodies. Immunoblotting and immunoprecipitation are sensitive and specific methods to detect these circulating antibodies. This article is protected by copyright. All rights reserved