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Updated: Jan 2, 2026

Technique of Conjunctival Biopsy and Direct Immunofluorescence for Diagnosing Mucous Membrane Pemphigoid
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IgA pemphigus: A systematic review.

Khalaf Kridin1, Payal M Patel2, Virginia A Jones2

  • 1Department of Dermatology, Rambam Health Care Campus, Haifa, Israel.

Journal of the American Academy of Dermatology
|December 9, 2019
PubMed
Summary
This summary is machine-generated.

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IgA pemphigus often presents as vesiculopustular eruptions and circinate plaques. This systematic review synthesizes data on IgA pemphigus, highlighting common clinical features and treatments.

Area of Science:

  • Dermatology
  • Immunology
  • Autoimmune Blistering Diseases

Background:

  • IgA pemphigus (IgAP) is a rare autoimmune blistering disease.
  • Limited large-scale studies exist on IgAP's clinical, histologic, and immunopathologic characteristics.

Purpose of the Study:

  • To conduct a systematic review synthesizing available data on IgA pemphigus.
  • To consolidate information on the epidemiology, clinical presentation, histology, and immunology of IgAP.

Main Methods:

  • Systematic literature review of MEDLINE, Embase, and Web of Science databases.
  • Inclusion of case reports and case series of patients diagnosed with IgA pemphigus.

Main Results:

  • 119 studies with 137 IgAP patients were analyzed (mean age 51.5 years).
Keywords:
IgA pemphigusimmunoreactivitysystematic review

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  • Common presentations included vesicles (80.8%), pustules (75.0%), circinate plaques (63.6%), and pruritus (65.6%).
  • Intercellular IgA deposition was found in 97.0% of patients; IgA gammopathy and ulcerative colitis were noted in 9.5% and 6.6%, respectively. Oral dapsone and corticosteroids were common treatments.
  • Conclusions:

    • IgA pemphigus diagnosis should be considered in patients with vesiculopustular eruptions and circinate plaques, particularly with truncal and extremity involvement.
    • Findings are primarily derived from case reports and small series, indicating a need for larger studies.