Direct and indirect immunofluorescence staining patterns in the diagnosis of paraneoplastic pemphigus

A. M. Poot*, J. . Siland, M. F. Jonkman, H. H. Pas, G. F. H. Diercks

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review

17 Citations (Scopus)


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.

Original languageEnglish
Pages (from-to)912-915
Number of pages4
JournalThe British journal of dermatology
Issue number4
Early online date11-Nov-2015
Publication statusPublished - Apr-2016



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