Funding sources None.
Clinical and Laboratory Investigations
Laboratory diagnosis of paraneoplastic pemphigus
Article first published online: 31 OCT 2013
© 2013 British Association of Dermatologists
British Journal of Dermatology
Volume 169, Issue 5, pages 1016–1024, November 2013
How to Cite
Poot, A.M., Diercks, G.F.H., Kramer, D., Schepens, I., Klunder, G., Hashimoto, T., Borradori, L., Jonkman, M.F. and Pas, H.H. (2013), Laboratory diagnosis of paraneoplastic pemphigus. British Journal of Dermatology, 169: 1016–1024. doi: 10.1111/bjd.12479
Conflicts of interest None declared.
- Issue published online: 31 OCT 2013
- Article first published online: 31 OCT 2013
- Accepted manuscript online: 25 JUN 2013 04:36AM EST
- Manuscript Accepted: 15 JUN 2013
Paraneoplastic pemphigus (PNP) is a multiorgan disease characterized by antibodies against plakins, desmogleins and the α2-macroglobulin-like-1 (A2ML1) protein, in association with an underlying neoplasm. Accurate diagnosis relies on the demonstration of these autoantibodies in serum.
To evaluate the value of different laboratory techniques in the serological diagnosis of PNP.
We performed immunoblotting, envoplakin (EP) enzyme-linked immunosorbent assay (ELISA), indirect immunofluorescence (IIF) on rat bladder, radioactive immunoprecipitation and a nonradioactive combined immunoprecipitation-immunoblot assay. Additional assays included BP180 ELISA and BP230 ELISA. We included the sera of 19 patients with PNP and 40 control subjects.
The sensitivities were 63% for anti-EP ELISA, 74% for rat bladder IIF, 89% for immunoblotting, 95% for radioactive immunoprecipitation and 100% for nonradioactive immunoprecipitation. Specificities ranged from 86% to 100%. The BP180 and BP230 ELISAs had low sensitivity and specificity for PNP. The combination of rat bladder IIF and immunoblot showed 100% sensitivity and specificity. The analysis of sequential PNP sera showed that antibody titres may decrease over time, possibly resulting in negative outcomes for EP ELISA and rat bladder IIF studies.
The detection of autoantibodies against EP and periplakin, or A2ML1 by immunoprecipitation is most sensitive for PNP. The combination of rat bladder IIF and immunoblotting is equally sensitive and highly specific, and represents an alternative valuable and relatively easy approach for the serological diagnosis of PNP.