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A.M. Poot, G.F.H. Diercks, D. Kramer, I. Schepens, G. Klunder, T. Hashimoto, L. Borradori, M.F. Jonkman and H.H. Pas Laboratory diagnosis of paraneoplastic pemphigus British Journal of Dermatology 169

Version of Record online: 31 OCT 2013 | DOI: 10.1111/bjd.12479

What's already known about this topic?

  • Paraneoplastic pemphigus (PNP) is a severe autoimmune disease characterized by anti plakin and anti-α2 macroglobulin-like 1 (A2ML1) autoantibodies.
  • Its laboratory diagnosis is based on demonstrating the presence of these autoantibodies in serum.

What does this study add?

  • This study shows the diagnostic value of an array of laboratory techniques for PNP.
  • We confirm that immunoprecipitation studies are the most sensitive, partly because of the detection of anti-A2ML1 antibodies.
  • Rat bladder indirect immunofluorescence in combination with immunoblotting provides an easier and accurate alternative to immunoprecipitation.
  • Envoplakin ELISA is of lower sensitivity.

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