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

Autor: Gilles F. H. Diercks, Marcel F. Jonkman, J. . Siland, Angelique Poot, Hendrikus Pas
Přispěvatelé: Translational Immunology Groningen (TRIGR), Microbes in Health and Disease (MHD)
Jazyk: angličtina
Rok vydání: 2016
Předmět:
Zdroj: The British journal of dermatology, 174(4), 912-915. Wiley
ISSN: 1365-2133
0007-0963
DOI: 10.1111/bjd.14282
Popis: 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.
Databáze: OpenAIRE