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Paraneoplastic pemphigus: a review

A Sklavounou1, G Laskaris

  • 1Department of Oral Medicine, Dental School, University of Athens, Greece.

Oral Oncology
|February 4, 1999
PubMed
Summary
This summary is machine-generated.

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Paraneoplastic pemphigus (PNP) is a rare autoimmune disorder often linked to lymphoid cancers. Most patients experience severe oral and skin lesions with limited treatment response.

Area of Science:

  • Autoimmune diseases
  • Dermatology
  • Oncology

Background:

  • Paraneoplastic pemphigus (PNP) is a distinct autoimmune blistering disease.
  • It differs from pemphigus vulgaris and foliaceus in clinical, histological, and immunological aspects.
  • PNP is associated with underlying malignancies, predominantly lymphoid neoplasms.

Purpose of the Study:

  • To review 18 reported cases of paraneoplastic pemphigus.
  • To discuss the clinical presentation, diagnostic challenges, and associated neoplasms in PNP.
  • To highlight treatment outcomes in patients with PNP.

Main Methods:

  • Review of 18 previously reported cases of paraneoplastic pemphigus.
  • Analysis of clinical, histological, and immunological data.

Related Experiment Videos

  • Discussion of associated malignancies and treatment responses.

    • Main Results:

    • The review included 18 cases of PNP.
    • Patients presented with polymorphic, extensive, and persistent skin and mucosal lesions.
    • The majority of associated malignancies were of lymphoid origin and preceded PNP onset.
    • All patients exhibited oral and cutaneous lesions.
    • A significant proportion (66.6%) showed a poor response to therapy.

    Conclusions:

    • Paraneoplastic pemphigus is a severe autoimmune disorder associated with neoplasms.
    • Early diagnosis and recognition of associated malignancies are crucial.
    • Treatment of PNP often proves challenging, with limited efficacy in many cases.