Coexistence of IgA antibodies to desmogleins 1 and 3 in pemphigus vulgaris, pemphigus foliaceus and paraneoplastic pemphigus

Mentink, Leon F.; Jong, M. C. J. M. de; Kloosterhuis, Guus J.; Zuiderveen, J.; Jonkman, Marcel F.; Pas, Hendrikus

doi:10.1111/j.1365-2133.2006.07717.x

Cited by 41 publications

(47 citation statements)

References 27 publications

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“…Similar to our patient, subcorneal pustular dermatoses without anti-Dsc1 IgA have been previously reported. 16,17 Clearly, as more sensitive detection methods become available, 9,18 the complexity of IGAP and pemphigus-related phenotypes in general is expected to further unfold. Further underscoring the complexity of IGAP, several cases have been reported with positive direct and indirect immunofluorescence evidence for IgA autoantibodies, but no reactivity to either Dsc or Dsg, [13][14][15] suggesting the existence of other, not yet identified, cell surface autoantigens in IGAP.…”

Section: Discussionmentioning

confidence: 99%

“…[10][11][12] A review of the literature indicates that SPD-like features can be associated with IgA antibodies targeting autoantigens other than Dsc1, whereas IgA reactivity against Dsc1 does not necessarily result in subcorneal pustule formation. Negative direct immunofluorescence can rarely be found in pemphigus vulgaris, but is certainly more common in IGAP, 17 presumably owing to the fact that IgA immune complexes are less stable and more sensitive to degradation. Conversely, IgA autoantibodies are far more common in apparently 'typical' IgG-mediated pemphigus vulgaris than generally assumed, as shown in two large studies.…”

Section: Discussionmentioning

confidence: 99%

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The expanding spectrum ofIgApemphigus: a case report and review of the literature

et al. 2014

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IgA pemphigus (IGAP) is a rare, distinct variant of pemphigus, currently classified, depending upon the histological features, immunofluorescence staining pattern and autoantibody profile, into two types: subcorneal pustular dermatosis (SPD) and intraepidermal neutrophilic IgA dermatosis. In a patient with a widespread blistering disease of the skin resembling SPD-type IGAP, we demonstrate the coexistence of IgA reactivity to both epidermal (desmocollins 2 and 3) and basement membrane-associated (BP180) proteins, suggesting the coexistence of atypical IGAP and linear IgA bullous dermatosis, respectively. This case, together with 20 previous reports of atypical IGAP, underscores the limitations of current classification schemes. Therefore, we suggest reclassifying these cases under the general term 'IGAP spectrum'.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

The expanding spectrum ofIgApemphigus: a case report and review of the literature

et al. 2014

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“…Mentink et al tested the sera of 100 cases of pemphigus patients (34 PF, 58 PV and 8 PNP) in both anti-Dsg-1 and 3 IgA ELISA tests and 54 sera were found to have IgA to one or both Dsgs. 131 They also found that more than half of the cases that showed IgA anti-Dsg in the ELISA presented negative staining for IgA in IIF and/or DIF. The ELISA thereby seems a more sensitive assay than IIF analysis for detecting anti-Dsg IgA antibodies.…”

Section: Definition and Epidemiologymentioning

confidence: 96%

“…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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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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“…Circulating IgG 2 and IgG 4 subclass autoantibodies were also observed in 60% and 23.3%, respectively, of healthy relatives [101]. Additionally, some PV patients develop IgA and IgE classes of Dsg 3 antibodies [113,114]. …”

Section: Pemphigus Autoantibodiesmentioning

confidence: 99%

Pemphigus autoimmunity: Hypotheses and realities

2011

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The goal of contemporary research in pemphigus vulgaris and pemphigus foliaceus is to achieve and maintain clinical remission without corticosteroids. Recent advances of knowledge on pemphigus autoimmunity scrutinize old dogmas, resolve controversies, and open novel perspectives for treatment. Elucidation of intimate mechanisms of keratinocyte detachment and death in pemphigus has challenged the monopathogenic explanation of disease immunopathology. Over 50 organ-specific and non-organ-specific antigens can be targeted by pemphigus autoimmunity, including desmosomal cadherins and other adhesion molecules, PERP cholinergic and other cell membrane (CM) receptors, and mitochondrial proteins. The initial insult is sustained by the autoantibodies to the cell membrane receptor antigens triggering the intracellular signaling by Src, epidermal growth factor receptor kinase, protein kinases A and C, phospholipase C, mTOR, p38 MAPK, JNK, other tyrosine kinases, and calmodulin that cause basal cell shrinkage and ripping desmosomes off the CM. Autoantibodies synergize with effectors of apoptotic and oncotic pathways, serine proteases, and inflammatory cytokines to overcome the natural resistance and activate the cell death program in keratinocytes. The process of keratinocyte shrinkage/detachment and death via apoptosis/oncosis has been termed apoptolysis to emphasize that it is triggered by the same signal effectors and mediated by the same cell death enzymes. The natural course of pemphigus has improved due to a substantial progress in developing of the steroid-sparing therapies combining the immunosuppressive and direct anti-acantholytic effects. Further elucidation of the molecular mechanisms mediating immune dysregulation and apoptolysis in pemphigus should improve our understanding of disease pathogenesis and facilitate development of steroid-free treatment of patients.

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Coexistence of IgA antibodies to desmogleins 1 and 3 in pemphigus vulgaris, pemphigus foliaceus and paraneoplastic pemphigus

Cited by 41 publications

References 27 publications

The expanding spectrum ofIgApemphigus: a case report and review of the literature

The expanding spectrum ofIgApemphigus: a case report and review of the literature

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

Pemphigus autoimmunity: Hypotheses and realities

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