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Cicatricial pemphigoid: IgA and IgG autoantibodies target epitopes on both intra‐ and extracellular domains of bullous pemphigoid antigen 180
Author(s) -
Schmidt E.,
Skrobek C.,
Kromminga A.,
Hashimoto T.,
Messer G.,
Bröcker EB.,
Yancey K.B.,
Zillikens D.
Publication year - 2001
Publication title -
british journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.304
H-Index - 179
eISSN - 1365-2133
pISSN - 0007-0963
DOI - 10.1046/j.1365-2133.2001.04471.x
Subject(s) - autoantibody , bullous pemphigoid , cicatricial pemphigoid , pemphigoid , epitope , antigen , antibody , immunology , medicine , ectodomain , recombinant dna , chemistry , biochemistry , receptor , gene
Background Cicatricial pemphigoid (CP) is an autoimmune subepidermal blistering disease where autoantibodies target various components of the dermal–epidermal junction, including the bullous pemphigoid antigen 180 (BP180). Objective We determined the exact specificity of circulating IgG and IgA autoantibodies to BP180 in a large number of CP patients. Methods Twenty‐six consecutive CP sera were analysed by Western blotting using a panel of cell‐derived and recombinant proteins covering the entire BP180 molecule. Results Circulating autoantibodies were detected in all CP sera. Seven sera reacting with laminin‐5 were excluded from further analyses; the remaining 19 sera recognized BP180, including six sera (32%) that showed only IgA reactivity to this protein. With the combined use of the soluble BP180 ectodomain (LAD‐1) and recombinant BP180 NC16A, 16 of these 19 CP sera (84%) targeted BP180. IgG reactivity was preferentially found against NC16A, whereas IgA antibodies predominantly recognized LAD‐1. Thirty‐two per cent of the BP180‐reative sera revealed reactivity with the intracellular domain of this protein. Conclusions Our findings demonstrate that autoantibodies in CP target epitopes on both extra‐ and intracellular domains of BP180 and highlight the importance of testing for both IgG and IgA reactivity in these patients' sera.