Related Experiment Videos
Paraneoplastic pemphigus: report of a case
S Izaki1, Y Yoshizawa, T Hashimoto
1Department of Dermatology, Saitama Medical Center, Saitama Medical School, Japan.
The Journal of Dermatology
|June 1, 1996
Summary
This study reports a rare case of paraneoplastic pemphigus in Japan. Treatment with plasmapheresis led to significant improvement in extensive mucocutaneous lesions and reduced autoantibody levels.
Area of Science:
- Dermatology
- Immunology
- Oncology
Background:
- Paraneoplastic pemphigus is a rare autoimmune blistering disease associated with lymphoproliferative disorders.
- Diagnosis can be challenging due to overlapping clinical and histological features with other mucocutaneous diseases.
Observation:
- A 56-year-old male with chronic lymphocytic leukemia presented with extensive erosive mucocutaneous lesions.
- Histopathology revealed acantholysis, and immunofluorescence showed IgG and C3 deposition in the intercellular space and at the dermo-epidermal junction.
Findings:
- Serum autoantibodies targeted human epidermal proteins of 190-250 kD, confirmed by Western blotting and immunoprecipitation.
- These findings strongly supported a diagnosis of paraneoplastic pemphigus.
Implications:
- Plasmapheresis therapy resulted in re-epithelialization of lesions and a marked decrease in autoantibody titers.
- This case highlights the importance of recognizing paraneoplastic pemphigus in patients with hematologic malignancies and mucocutaneous lesions, even in regions where it is rarely reported.