To describe a patient with severe, recalcitrant mucous membrane erosions and chronic lymphocytic leukemia. We present a case report and a literature review of paraneoplastic pemphigus. Immunofluorescence studies and immunoprecipitation confirmed the presence of autoantibodies characteristic of paraneoplastic pemphigus in the patient's serum. 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. The clinical course of paraneoplastic pemphigus tends to be rapid and fatal despite immunosuppressive therapy. Long-term survival is uncommon.
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