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Two Brazilian Cases of IgA Pemphigus
Author(s) -
Oliveira Juliana Pedroso,
Gabbi Tatiana Villas Boas,
Hashimoto Takashi,
Aoki Valéria,
Santi Cláudia G.,
Maruta Celina W.,
Rivitti Evandro A.,
Reis Vitor M.S.
Publication year - 2003
Publication title -
the journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.9
H-Index - 65
eISSN - 1346-8138
pISSN - 0385-2407
DOI - 10.1111/j.1346-8138.2003.tb00343.x
Subject(s) - pemphigus , desmoglein , medicine , antibody , dermatology , immunofluorescence , paraneoplastic pemphigus , immunology , pathology , autoantibody
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.