Paraneoplastic pemphigus: a case of prolonged survival.

Perniciaro, C; Kuechle, M K; Colón-Otero, G; Raymond, M G; Spear, K L; Pittelkow, M R · Mayo Clin Proc · 1994

case_report · Level V

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Abstract

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.

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