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Paraneoplastic pemphigus: the first case report from Japan
Y Nishibori1, T Hashimoto, A Ishiko
1Department of Dermatology, Keio University School of Medicine, Tokyo, Japan.
Dermatology (Basel, Switzerland)
|January 1, 1995
Summary
Paraneoplastic pemphigus is a rare autoimmune blistering disease linked to cancer. This case study details a Japanese patient with non-Hodgkin
Area of Science:
- Dermatology
- Autoimmunology
- Oncology
Background:
- Paraneoplastic pemphigus (PP) is a rare, severe autoimmune blistering disease.
- It is strongly associated with an underlying lymphoid or epithelial malignancy.
- PP presents with painful mucosal erosions and polymorphic skin lesions.
Observation:
- A 54-year-old Japanese male, previously treated for non-Hodgkin's lymphoma, presented with severe stomatitis, conjunctivitis, and blistering.
- Histopathology revealed suprabasal clefts, acantholysis, and keratinocyte necrosis.
- Direct immunofluorescence showed IgG on keratinocyte cell surfaces and C3 at the basement membrane zone.
Findings:
- Indirect immunofluorescence detected autoantibodies targeting keratinocyte and transitional epithelial cell surfaces.
- Immunoprecipitation identified antibodies against 250-kD, 230-kD, 210-kD, and 170-kD proteins.
- These findings confirmed the diagnosis of paraneoplastic pemphigus.
Implications:
- This case represents the first reported instance of paraneoplastic pemphigus in Japan.
- Highlights the importance of recognizing PP in patients with a history of malignancy and mucocutaneous lesions.
- Contributes to the understanding of PP's clinical and immunopathological spectrum.