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Childhood bullous pemphigoid associated with IgA antibodies against BP180 or BP230 antigens
Author(s) -
Ana Arechalde,
Ralph P. Braun,
Calza Am,
Michael Hertl,
Liliane Didierjean,
Saurat Jh,
Luca Borradori
Publication year - 1999
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.1365-2133.1999.02618.x
Subject(s) - bullous pemphigoid , antibody , antigen , immunology , ectodomain , medicine , immunoglobulin a , immunoglobulin g , receptor
Linear IgA bullous dermatosis (LABD) comprises a heterogeneous group of subepidermal blistering disorders characterized by in situ bound IgA antibodies in epidermal basement membrane. We report three children presenting clinical and immunopathological features characteristic of LABD. By immunoblotting, the three patients' sera contained IgA antibodies that reacted against the bullous pemphigoid (BP) antigen 180 and or BP230, molecular markers for BP. In addition, IgG antibodies directed against the ectodomain of BP180 were detected by an enzyme‐linked immunosorbent assay using a eukaryotic recombinant form of BP180. Consistent with recent studies suggesting that the LABD antigen 1, the predominant autoantigen of LABD, is either a proteolytic product of BP180 or an isoform of the BP180 gene, our findings indicate that a subset of children with features of LABD have a distinct form of BP associated with an IgA response.