title: Two Brazilian cases of IgA pemphigus.
abstract: IgA pemphigus is a rare, neutrophilic, acantholytic skin disorder with approximately 70 cases described in the literature. We report two patients with the subcorneal pustular dermatosis (SPD) type of IgA pemphigus. Initially, both patients were misdiagnosed as subcorneal pustular dermatosis of Sneddon and Wilkinson. The correct diagnosis was only made after detecting intercellular IgA depositions in the epidermis by direct immunofluorescence. Immunoblotting (IB) of normal human epidermal extracts, performed on both sera, was negative for Dsg 1, Dsg 3, BP 230, BP 180, 210 kDa envoplakin, and 190 kDa periplakin. ELISA for desmogleins (Dsg 1 and Dsg 3) showed that neither of the cases had IgA antibodies to Dsg. The c-DNA transfection test for desmocollins (Dsc) revealed that the IgA antibodies of both patients reacted with desmocollin 1. This result supports the hypothesis that the autoantigen in SPD type IgA pemphigus is desmocollin 1.
authors.affiliation: Department of Dermatology of University of Sao Paulo, Brazil.
authors.forename: Juliana Pedroso
authors.initials: JP
authors.lastname: de Oliveira
authors.forename: Tatiana Villas Boas
authors.initials: TV
authors.lastname: Gabbi
authors.forename: Takashi
authors.initials: T
authors.lastname: Hashimoto
authors.forename: Valéria
authors.initials: V
authors.lastname: Aoki
authors.forename: Cláudia G
authors.initials: CG
authors.lastname: Santi
authors.forename: Celina W
authors.initials: CW
authors.lastname: Maruta
authors.forename: Evandro A
authors.initials: EA
authors.lastname: Rivitti
authors.forename: Vitor M S
authors.initials: VM
authors.lastname: Reis
mesh_terms: D000328:Adult; D001324:Autoantigens / Q000276:immunology; D002648:Child; D018076:DNA, Complementary / Q000032:analysis; D051187:Desmocollins; D003937:Diagnosis, Differential; D004797:Enzyme-Linked Immunosorbent Assay; D005260:Female; D006801:Humans; D007070:Immunoglobulin A / Q000276:immunology*; D008297:Male; D008562:Membrane Glycoproteins / Q000276:immunology*; D010392:Pemphigus / Q000175:diagnosis* / Q000473:pathology
chemical_list: D001324:Autoantigens; D018076:DNA, Complementary; C081560:DSC1 protein, human; D051187:Desmocollins; D007070:Immunoglobulin A; D008562:Membrane Glycoproteins