[Paraneoplastic pemphigus: Retrospective study of a case series]

Area of Science:

• Dermatology
• Autoimmune Diseases
• Oncology

Background:

• Paraneoplastic pemphigus (PNP) is a rare autoimmune blistering disease.
• It presents with diverse mucocutaneous symptoms linked to underlying neoplasia.
• Diagnosis is challenging due to varied clinical, histological, and immunological features.

Purpose of the Study:

• To describe the clinical, histological, and immunological characteristics of PNP patients.
• To evaluate the prognosis and survival rates in a cohort of PNP patients.
• To discuss diagnostic criteria and potential factors influencing prognosis.

Main Methods:

• Retrospective analysis of PNP patient records from 2000-2015.
• Review of clinical presentations, histological findings, and immunofluorescence results.
• Assessment of neoplasia association and patient survival.

Main Results:

• Seven patients with PNP were identified, associated with lymphoma, melanoma, or carcinomas.
• Common findings included epidermal acantholysis, keratinocytic necrosis, and interface dermatitis.
• Direct immunofluorescence showed intercellular IgG/C3 deposits; indirect IF was positive in 4/6 patients.
• One-year survival rate was 85.7%.

Conclusions:

• PNP presentations were polymorphic, overlapping with classical pemphigus.
• Survival in this series exceeded literature data, potentially due to fortuitous neoplasia links.
• Revised diagnostic criteria for PNP are needed for accurate diagnosis and trials.