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Granulocyte-dependent Autoantibody-induced Skin Blistering

Granulocyte-dependent Autoantibody-induced Skin Blistering

Published on: October 12, 2012

Paraneoplastic pemphigus: a short review.

Marta Wieczorek¹, Annette Czernik²

¹Clinical Department of Dermatology, Central Clinical Hospital of the Ministry of the Interior and Administration, Warsaw, Poland.

Clinical, Cosmetic and Investigational Dermatology

|October 13, 2016

Summary

Paraneoplastic pemphigus (PNP) is a rare, severe autoimmune blistering disease linked to cancer. Early diagnosis and treatment are crucial for managing this condition and improving patient outcomes.

Keywords:

direct and indirect immunofluorescence mucositis paraneoplastic pemphigus rituximab

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Area of Science:

Autoimmune diseases
Dermatology
Oncology

Background:

Paraneoplastic pemphigus (PNP) is a rare, life-threatening autoimmune blistering disease associated with an underlying malignancy.
First described in 1990, PNP affects multiple organ systems, presenting with diverse lesions including severe oral mucosal lesions, skin eruptions, and pulmonary involvement.

Approach:

Diagnosis involves identifying systemic complications, skin biopsies for histopathology and immunofluorescence, and serum immunological studies to detect autoantibodies.
Key autoantibodies target antigens like desmoplakin I, envoplakin, and periplakin.

Key Points:

PNP exhibits at least five clinical variants and variable histological findings.
Unlike other pemphigus forms, PNP can affect the gastrointestinal and respiratory tracts.
Early and consistent finding is severe stomatitis.

Conclusions:

Treatment is challenging; surgical excision of benign neoplasms offers the best outcomes.
High-dose corticosteroids with steroid-sparing agents are first-line treatments; rituximab and IVIg are used for treatment failures.
Prognosis is generally poor due to tumor progression and infectious complications from immunosuppression, which is the leading cause of death.