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.

Immunoglobulin A pemphigus associated with immunoglobulin A gammopathy and lung cancer

“…In the published work, more than 70 cases of IgA pemphigus have been reported, with prevalence of SPD type. 1,11,16 IgA pemphigus has been rarely associated with benign or malignant monoclonal IgA paraprotein, usually discovered at the time of presentation; this association was found exclusively in the SPD subtype. [1][2][3]10,11 IgA pemphigus has been reported with myeloma and B-cell lymphoma in the past, 2 and for the first time with lung cancer as in our case.…”

Imunofluorescência direta e indireta

“…[19][20] Subepidermal bullous dermatosis Bullous pemphigoid IIF: It shows circulating antibodies of the anticollagen XVII (BP180) IgG class in 70% of the patients. It appears that there is no correlation between antibody titers and the extension or activity of the disease.…”

IgA Pemphigus in a Child – a Case Report

“…IGAP is an extremely rare condition in children -detailed PubMed search revealed only 9 cases, of whom 3 were classified as SPD type (two girls and one boy), 2 were IEN type (one girl and one boy), one was classified as pemphigus vegetans variant IGAP (one boy) and the other 3 cases were unclassified (three girls) (5,6,7,8,9,10,11,12,13). The youngest reported patient was a 1-month-old girl (9).…”

“…IgA pemphigus is a rare, neutrophilic, acantholytic skin disorder that usually occurs in middle-aged and elderly persons (approximately 70 cases have been reported in the literature); the average age of onset is 48 years (6). IGAP is an extremely rare condition in children -detailed PubMed search revealed only 9 cases, of whom 3 were classified as SPD type (two girls and one boy), 2 were IEN type (one girl and one boy), one was classified as pemphigus vegetans variant IGAP (one boy) and the other 3 cases were unclassified (three girls) (5,6,7,8,9,10,11,12,13).…”