Article ID Journal Published Year Pages File Type
1966359 Clinica Chimica Acta 2009 6 Pages PDF
Abstract

IntroductionIn paraneoplastic pemphigus (PNP), indirect immunofluorescence microscopy using rat bladder sections is widely used to search for circulating autoantibodies which are predominantly directed against envoplakin and periplakin. A sensitive and specific detection system for envoplakin/periplakin-specific autoantibodies is not yet available.MethodsOverlapping fragments spanning envoplakin and periplakin, respectively, were analyzed for their ability to bind PNP-specific autoantibodies by immunoblotting and ELISA in sera from patients with PNP (n = 31), pemphigus vulgaris (n = 30), and bullous pemphigoid (n = 50) as well as healthy volunteers (n = 140). The results were compared with those obtained by immunoblotting of extract of cultured human keratinocytes.ResultsImmunoblot analysis revealed that most sera contained antibodies against the N-termini of both plakins as well as against the C-terminus of envoplakin. By ELISA, reactivities against envoplakin1–481, envoplakin1626–2033, and periplakin1–324 were found in 25 (80.6%), 25 (80.6%), and 23 (74.2%) PNP sera, and in 1 (1.2%), 3 (3.7%), and 2 (2.5%) control sera, respectively.ConclusionsThe new ELISA based on an N-terminal fragment of envoplakin shows a high diagnostic accuracy to detect circulating autoantibodies in PNP. It is easy to be setup and standardized and can help to differentiate PNP patients from those with pemphigus vulgaris.

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