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Clin Immunol. 2010 Apr;135(1):99-107. doi: 10.1016/j.clim.2009.12.010. Epub 2010 Jan 25.

T and B cells target identical regions of the non-collagenous domain 1 of type VII collagen in epidermolysis bullosa acquisita.

Author information

1
Department of Dermatology and Allergology, Philipps University Deutschhausstrasse 9, D-35033 Marburg, Germany. Ralf.Mueller@uk-sh.de

Abstract

Epidermolysis bullosa acquisita (EBA) is a severe immunobullous disease and is caused by IgG against type VII collagen (Col VII) of anchoring fibrils. In this study, utilizing ELISA and immunoblot, 13/15 EBA sera but 0/20 bullous pemphigoid sera and 0/30 healthy control sera showed IgG reactivity with distinct recombinant subregions of the non-collagenous domain 1 (NC1) of Col VII. In two EBA patients, IgG titers against Col VII-NC1 were grossly correlated to clinical disease activity. Moreover, Col VII-reactive T cells were identified in a representative EBA patient which recognized identical subdomains of Col VII-NC1. These findings strongly suggest that (1) the Col VII-NC1 ELISA is a powerful tool for making the diagnosis of EBA, (2) Col VII-specific IgG grossly relates to disease activity and (3) IgG reactivity is associated with T cell recognition of identical subdomains of Col VII-NC1.

PMID:
20093095
DOI:
10.1016/j.clim.2009.12.010
[Indexed for MEDLINE]

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