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Paraneoplastic pemphigus: a case of prolonged survival
C Perniciaro1, M K Kuechle, G Colón-Otero
1Department of Dermatology, Mayo Clinic Jacksonville, Florida 32224.
Objective:
To describe a patient with severe, recalcitrant mucous membrane erosions and chronic lymphocytic leukemia.
Design:
We present a case report and a literature review of paraneoplastic pemphigus.
Material And Methods:
Immunofluorescence studies and immunoprecipitation confirmed the presence of autoantibodies characteristic of paraneoplastic pemphigus in the patient's serum.
Results:
Our patient lived almost 8 years after the onset of paraneoplastic pemphigus, the longest time that anyone with this disease is known to have survived.
Conclusion:
The clinical course of paraneoplastic pemphigus tends to be rapid and fatal despite immunosuppressive therapy. Long-term survival is uncommon.