title: Childhood bullous pemphigoid associated with IgA antibodies against BP180 or BP230 antigens.
abstract: 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.
authors.affiliation: Department of Dermatology, University Hospital Geneva, 24, rue Micheli-du-Crest, CH-1211 Geneva 14, Switzerland.
authors.forename: A
authors.initials: A
authors.lastname: Arechalde
authors.forename: R P
authors.initials: RP
authors.lastname: Braun
authors.forename: A M
authors.initials: AM
authors.lastname: Calza
authors.forename: M
authors.initials: M
authors.lastname: Hertl
authors.forename: L
authors.initials: L
authors.lastname: Didierjean
authors.forename: J H
authors.initials: JH
authors.lastname: Saurat
authors.forename: L
authors.initials: L
authors.lastname: Borradori
mesh_terms: D000888:Antibodies, Anti-Idiotypic; D001324:Autoantigens / Q000276:immunology*; D002648:Child; D002675:Child, Preschool; D005260:Female; D005455:Fluorescent Antibody Technique; D006801:Humans; D015151:Immunoblotting; D007070:Immunoglobulin A / Q000276:immunology*; D008297:Male; D024041:Non-Fibrillar Collagens; D010391:Pemphigoid, Bullous / Q000175:diagnosis / Q000276:immunology*; D000097805:Collagen Type XVII
chemical_list: D000888:Antibodies, Anti-Idiotypic; D001324:Autoantigens; D007070:Immunoglobulin A; D024041:Non-Fibrillar Collagens; D000097805:Collagen Type XVII