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Is pemphigus herpetiformis an entity?

E Maciejowska1, S Jablonska, T Chorzelski

  • 1Department of Dermatology, Warsaw School of Medicine, Poland.

International Journal of Dermatology
|November 1, 1987
PubMed
Summary
This summary is machine-generated.

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Pemphigus herpetiformis, initially misdiagnosed, shows distinct IgG intercellular staining. Treatment often requires combined immunosuppressants like prednisone and cyclophosphamide for effective management.

Area of Science:

  • Dermatology
  • Immunodermatology
  • Autoimmune Blistering Diseases

Background:

  • Pemphigus herpetiformis is a rare autoimmune blistering disease.
  • It can be initially misdiagnosed as dermatitis herpetiformis, IgA linear bullous dermatosis, or bullous pemphigoid.
  • Understanding its distinct clinical, histological, and immunofluorescence features is crucial for accurate diagnosis and management.

Purpose of the Study:

  • To characterize the clinical, histological, and immunofluorescence findings in patients with pemphigus herpetiformis.
  • To evaluate the therapeutic responses to various treatment regimens.
  • To analyze the disease course and potential evolution of skin lesions.

Main Methods:

  • Clinical examination of 15 patients with pemphigus herpetiformis.

Related Experiment Videos

  • Histopathological analysis of skin biopsies.
  • Direct and indirect immunofluorescence studies using various substrates.
  • Assessment of treatment efficacy with sulfones, prednisone, and cyclophosphamide.

    • Main Results:

    • Histology revealed eosinophilic spongiosis or acantholysis with microabscesses.
    • Direct immunofluorescence consistently showed intercellular IgG deposition.
    • Indirect immunofluorescence was positive in 5/15 patients.
    • Approximately 50% of patients responded to sulfones and prednisone; some required combined therapy with cyclophosphamide or higher prednisone doses.
    • Nine patients maintained pemphigus herpetiformis features upon relapse, while others showed pemphigus seborrheicus-foliaceus type lesions.

    Conclusions:

    • Pemphigus herpetiformis exhibits characteristic intercellular IgG staining on direct immunofluorescence.
    • Treatment often necessitates a combination of immunosuppressive agents.
    • The disease may evolve into other pemphigus subtypes during relapses.