A Case of IgG/IgA Pemphigus Presenting Malar Rash-like Erythema

Hosoda, Satomi; Suzuki, Masayuki; Komine, Mayumi; Murata, Satoru; Hashimoto, Takashi; Ohtsuki, Mamitaro

doi:10.2340/00015555-1258

Cited by 15 publications

(4 citation statements)

References 14 publications

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“…113 Since then we have found another 14 similar case reports. 114-126 Two other articles that studied the frequency of IgA antibodies in different bullous diseases 127 and the autoantigens recognized by IgA anti-keratinocyte cell surface antibodies both describe another six not previously reported cases presenting with intercellular IgG and IgA in the DIF. 60 Three other cases were also called IgG/ IgA pemphigus, despite presenting negative DIF 128 or only intercellular IgG by DIF (but both intercellular IgG and IgA by IIF) or only intercellular IgA by DIF (but both intercellular IgG and IgA by IIF).…”

Section: Igg/ Iga Pemphigusmentioning

confidence: 99%

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Non-classical forms of pemphigus: pemphigus herpetiformis, IgA pemphigus, paraneoplastic pemphigus and IgG/IgA pemphigus

Porro

Caetano

Maehara

et al. 2014

An. Bras. Dermatol.

122

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The pemphigus group comprises the autoimmune intraepidermal blistering diseases classically divided into two major types: pemphigus vulgaris and pemphigus foliaceous. Pemphigus herpetiformis, IgA pemphigus, paraneoplastic pemphigus and IgG/IgA pemphigus are rarer forms that present some clinical, histological and immunopathological characteristics that are different from the classical types. These are reviewed in this article. Future research may help definitively to locate the position of these forms in the pemphigus group, especially with regard to pemphigus herpetiformis and the IgG/ IgA pemphigus.

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Section: Igg/ Iga Pemphigusmentioning

confidence: 99%

“…60,63,72,114-131 Pruritus, pustules and annular lesions are present in almost half of the cases. Most of them do not show mucous lesions.…”

Section: Clinical Featuresmentioning

confidence: 99%

Non-classical forms of pemphigus: pemphigus herpetiformis, IgA pemphigus, paraneoplastic pemphigus and IgG/IgA pemphigus

Porro

Caetano

Maehara

et al. 2014

An. Bras. Dermatol.

122

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“…There has been a debate as to whether IgG/IgA pemphigus exists as a single distinct entity or a transitional phase along a spectrum of IgG pemphigus to IgA pemphigus. Studies have confirmed the role of IgG and of IgA to Dsg 1 and Dsg 3 as the predominant antibodies among these patients, but several studies have reported Dsc 1 and Dsc 3 as target antigens too [ 47 ]. One explanation for this heterogeneity, which is shown to be a characteristic of IgG/IgA pemphigus, is the epitope spreading phenomenon in which an inflammatory event releases new target antigens, exposes them to the immune system, and then induces subsequent autoimmunity to new related antigens [ 48 ].…”

Section: Non Classical Pemphigus Clinical Variantsmentioning

confidence: 90%

Pemphigus—The Crux of Clinics, Research, and Treatment during the COVID-19 Pandemic

et al. 2021

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Pemphigus is a rare autoimmune disease characterised by the production of pathogenic autoantibodies in response to different desmosome proteins. The pathophysiological process leads to the development of blisters and erosions on mucosal and/or skin surfaces. The classical clinical variants of pemphigus are pemphigus vulgaris and pemphigus foliaceus. A diagnostic delay is very common in pemphigus, especially among patients with mucosal involvement. However, in recent years we have witnessed considerably fewer patients with extensive mucocutaneous manifestations, since patients with oral lesions are referred to dermatologists to start the treatment much sooner than they had been previously. Among non-classical variants of pemphigus, unusual cases with discrepancies between autoantibody profiles and clinics challenge the “desmoglein compensation theory”. The identification of several other autoantigens that perform a role in the pathogenesis of different variants of pemphigus will progress immunodermatology towards an approach that will determine personalized pemphigus subtypes for each patient. Comorbidities among patients are primarily associated with the prolonged use of corticosteroids and other immunosuppressive agents. The SARS-CoV-2 pandemic raised concerns regarding the immunosuppressive effects of treatment and the risk of a more complicated COVID-19 infection, as well as on the ability to develop an adequate vaccine response.

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“…This profile was described in 35 patients (Supplementary Table 4) (8,10,38,(41)(42)(43)(44)(45)(46)(47)(48)(49)(50)(51)(52)(53). Age and sex were not always reported.…”

Section: Patients With Only Anti-desmocollin and Anti-desmoglein Autoantibodiesmentioning

confidence: 98%

Anti-Desmocollin Autoantibodies in Autoimmune Blistering Diseases

et al. 2021

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The presence of anti-desmocollin (Dsc) antibodies is rarely described in autoimmune blistering diseases patients. Moreover, several clinical phenotypes of pemphigus may be associated with these antibodies. In this review we analyze clinicopathological, immunologic and outcome features of anti-Dsc autoimmune blistering diseases patients, to improve their diagnosis and management. We conducted a systematic search of PubMed and Embase (1990-present) for studies reporting cases of autoimmune blistering diseases with anti-Dsc antibodies. We classified the selected patients as patients with exclusively anti-Dsc autoantibodies, and patients with anti-Dsc and other autoantibodies. Of 93 cases with anti-Dsc autoantibodies included, 38 (41%) had exclusively these antibodies. Only 18% of patients presented with the typical clinicopathological phenotype of pemphigus vulgaris or pemphigus foliaceous. Mucosal involvement was seen in approximately half of the patients. Up to 18% of cases were associated with neoplasms. Acantholysis was described in 54% of cases with histopathological information. Treatments and outcomes vary in the different clinical phenotypes. The presence of anti-Dsc antibodies must be suspected mainly in those patients with either atypical pemphigus, in special with clinical pustules, or in cases showing intraepithelial or dermal neutrophilic/eosinophilic infiltrate on histological examination and dual pattern by direct immunofluorescence examination.

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A Case of IgG/IgA Pemphigus Presenting Malar Rash-like Erythema

Cited by 15 publications

References 14 publications

Non-classical forms of pemphigus: pemphigus herpetiformis, IgA pemphigus, paraneoplastic pemphigus and IgG/IgA pemphigus

Non-classical forms of pemphigus: pemphigus herpetiformis, IgA pemphigus, paraneoplastic pemphigus and IgG/IgA pemphigus

Pemphigus—The Crux of Clinics, Research, and Treatment during the COVID-19 Pandemic

Anti-Desmocollin Autoantibodies in Autoimmune Blistering Diseases

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