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[Paraneoplastic pemphigus].

V Descamps1, S Belaich

  • 1Service de dermatologie, hôpital Bichat, Paris.

Presse Medicale (Paris, France : 1983)
|March 20, 1999
PubMed
Summary
This summary is machine-generated.

Paraneoplastic pemphigus is a rare autoimmune blistering disease associated with cancers, primarily hematologic malignancies. Diagnosis requires identifying autoantibodies against specific antigens, as clinical signs are polymorphous.

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Area of Science:

  • Dermatology
  • Autoimmunology
  • Oncology

Background:

  • Paraneoplastic pemphigus (PNP) is a rare, recently recognized autoimmune blistering disease.
  • Since its 1990 description, approximately 50 cases have been reported worldwide.
  • PNP is characterized by polymorphous mucocutaneous lesions and is associated with various cancers.

Purpose of the Study:

  • To summarize the clinical manifestations, associated cancers, diagnostic criteria, and pathogenesis of paraneoplastic pemphigus.
  • To highlight the challenges in diagnosing and treating this rare condition.

Main Methods:

  • Review of clinical signs, including skin and mucosal erosions, bullae, and urticarial lesions.
  • Identification of associated malignancies, predominantly hematologic disorders.

Related Experiment Videos

  • Analysis of diagnostic criteria, including histopathology, immunohistology, and serological methods like immunoprecipitation and immunoblotting to detect specific autoantibodies.

    • Main Results:

    • Clinical presentation is polymorphous, mimicking pemphigus vulgaris, pemphigoid, and erythema multiforme, with mucosal erosions being predominant.
    • Associated cancers are mainly hematologic diseases such as non-Hodgkin's lymphomas and chronic lymphoid leukemia.
    • Diagnostic confirmation relies on detecting autoantibodies targeting plakin components, desmoglein 3, and other antigens via immunoprecipitation or immunoblotting.

    Conclusions:

    • Paraneoplastic pemphigus is a distinct autoimmune paraneoplastic syndrome with a severe prognosis, often lacking a standardized treatment.
    • The pathogenesis is hypothesized to involve tumor-induced loss of tolerance to keratinocyte antigens.
    • Early diagnosis and management of the underlying neoplasm are crucial, although the disease course may not always correlate with cancer progression.