Paraneoplastic pemphigus: a case of prolonged survival.

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.