IgA pemphigus in a child

Caputo, Ruggero; Pistritto, G.; Giannì, E; Carminati, G.; Crupi, A. C.; Berti, Emilio; Gelmetti, Carlo; Croci, S.

doi:10.1016/s0190-9622(08)80392-1

Cited by 23 publications

(9 citation statements)

References 9 publications

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“…Table 1 summarizes the key clinical and microscopic features of our patient compared to the five other patients with childhood IgA pemhigus reported in the English‐language literature. Similar to our patient, cutaneous vesiculopustules and eosinophilia were common features in those described by Saurat et al (17), Caputo et al (18), and Teraki et al (19). Teraki et al (19) also described mucous membrane involvement, but did not identify IgG antibodies or a target antigen.…”

Section: Discussionsupporting

confidence: 91%

“…As with our patient, dapsone was often the first choice for therapy, and in some instances resulted in complete clearing of the lesions (19,20). In other patients, corticosteroids or other immune modulating drugs were needed to control the disease (17,18,21). Based on the reports in which outcome was indicated, the overall prognosis for children with IgA pemphigus seems benign, as four of five patients were able to gradually discontinue their therapies without relapsing.…”

Section: Discussionmentioning

confidence: 99%

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Atypical IgA/IgG Pemphigus Involving the Skin, Oral Mucosa, and Colon in a Child: A Novel Variant of IgA Pemphigus?

Bruckner

Fitzpatrick

Hashimoto

et al. 2005

Pediatric Dermatology

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Immune-mediated blistering diseases are rare in childhood, with the subset of IgA pemphigus being extremely uncommon. A child with a bullous disorder of the oral mucosa, skin, and colon is reported because of the unusual nature of the clinical and laboratory findings. Immunofluorescence studies demonstrated both intercellular epidermal and basement membrane zone deposition of IgA and IgG antibodies, as well as intercellular colonic deposition of IgA antibodies. IgA and IgG ELISA tests were positive for desmoglein 3 as the intercellular epidermal antigen. The generation of multiple antibodies against several potential antigenic targets suggests epitope spreading may play a role in this disease. We believe this represents a novel variant of IgA pemphigus.

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Section: Discussionsupporting

confidence: 91%

Section: Discussionmentioning

confidence: 99%

Atypical IgA/IgG Pemphigus Involving the Skin, Oral Mucosa, and Colon in a Child: A Novel Variant of IgA Pemphigus?

Bruckner

Fitzpatrick

Hashimoto

et al. 2005

Pediatric Dermatology

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“…It usually occurs in middle‐aged or elderly people and only seldom in childhood (12). To our knowledge, only seven cases in children have been reported so far (3, 11, 15–18), including our patient (case 2). The earliest age of onset was one month old (18).…”

Section: Discussionmentioning

confidence: 80%

“…Subsequently, multiple similar cases were described, and different names have been used, including intraepidermal neutrophilic IgA dermatosis (2–4); atypical neutrophilic dermatosis (5); intercellular IgA dermatosis (6); intercellular IgA vesiculopustular dermatosis (7); IgA herpetiform pemphigus (8) and IgA pemphigus foliaceus (9). The term “IgA Pemphigus” has been more often used since it was first proposed by Hodak et al (10) and Caputo et al (11) IgA pemphigus is a newly characterized group of autoimmune, intraepidermal, blistering diseases that exhibit a vesiculopustular eruption, neutrophil infiltration, acantholysis, and in vivo bound and circulating IgA autoantibodies that target cell surface components of the epidermis (12). Currently, IgA pemphigus has been classified into two distinct subtypes: subcorneal pustular dermatosis (SPD type) (1, 2, 5, 6, 10, 12, 19–27, 29–33, 35–39) and intraepidermal neutrophilic IgA dermatosis (IEN type) (3, 4, 6, 12, 14, 18–21, 27, 28, 33, 34, 38).…”

Section: Introductionmentioning

confidence: 99%

Two Brazilian Cases of IgA Pemphigus

Oliveira

Gabbi

Hashimoto

et al. 2003

The Journal of Dermatology

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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.

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“…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).…”

Section: Discussionmentioning

confidence: 99%

IgA Pemphigus in a Child – a Case Report

Lekić

Gajić-Veljić

Popadić

et al. 2017

Serbian Journal of Dermatology and Venereology

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IgA pemphigus (IGAP) is a rare autoimmune bullous disease characterized by IgA deposits on keratinocyte cell surfaces. The IGAP is classified into: 1) subcorneal pustular dermatosis (SPD) type, and 2) intraepidermal neutrophilic (IEN) IgA dermatosis type. So far, only 9 children with IGAP have been described in the literature, of whom only 3 with SPD type. We report a 3-year-old boy with SPD type of IGAP. Clinically, he presented with pruritic vesicles, pustules and erosions on the face, trunk, groin area, and extremities. Histopathology showed subcorneal pustules containing a few acantholytic cells. Direct immunofluorescence (DIF) test of Tzanck smear showed intercellular IgA deposits on the surface of the groups of epidermal cells. Oral dapsone and prednisone induced remission after two weeks; the treatment was discontinued 11 months later, and complete remission was achieved during 19 months without any treatment. Direct immunofluorescence of Tzanck smear is a simple, sensitive, rapid and non-aggressive test, very suitable for the diagnosis of IGAP in children.

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IgA pemphigus in a child

Cited by 23 publications

References 9 publications

Atypical IgA/IgG Pemphigus Involving the Skin, Oral Mucosa, and Colon in a Child: A Novel Variant of IgA Pemphigus?

Atypical IgA/IgG Pemphigus Involving the Skin, Oral Mucosa, and Colon in a Child: A Novel Variant of IgA Pemphigus?

Two Brazilian Cases of IgA Pemphigus

IgA Pemphigus in a Child – a Case Report

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