title: T and B cells target identical regions of the non-collagenous domain 1 of type VII collagen in epidermolysis bullosa acquisita.
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.
authors.affiliation: Department of Dermatology and Allergology, Philipps University Deutschhausstrasse 9, D-35033 Marburg, Germany. Ralf.Mueller@uk-sh.de
authors.forename: Ralf
authors.initials: R
authors.lastname: Müller
authors.forename: Christiane
authors.initials: C
authors.lastname: Dahler
authors.forename: Christian
authors.initials: C
authors.lastname: Möbs
authors.forename: Elke
authors.initials: E
authors.lastname: Wenzel
authors.forename: Rüdiger
authors.initials: R
authors.lastname: Eming
authors.forename: Gerald
authors.initials: G
authors.lastname: Messer
authors.forename: Andrea
authors.initials: A
authors.lastname: Niedermeier
authors.forename: Michael
authors.initials: M
authors.lastname: Hertl
mesh_terms: D001323:Autoantibodies / Q000276:immunology*; D001402:B-Lymphocytes / Q000276:immunology*; D015153:Blotting, Western; D003001:Cloning, Molecular; D015331:Cohort Studies; D024143:Collagen Type VII / Q000235:genetics / Q000276:immunology*; D004247:DNA / Q000737:chemistry / Q000235:genetics; D004797:Enzyme-Linked Immunosorbent Assay; D016107:Epidermolysis Bullosa Acquisita / Q000175:diagnosis / Q000276:immunology*; D000939:Epitopes / Q000276:immunology; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood / Q000276:immunology*; D016133:Polymerase Chain Reaction; D011994:Recombinant Proteins / Q000276:immunology; D013601:T-Lymphocytes / Q000276:immunology*
chemical_list: D001323:Autoantibodies; D024143:Collagen Type VII; D000939:Epitopes; D007074:Immunoglobulin G; D011994:Recombinant Proteins; D004247:DNA