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Paraneoplastic pemphigus: a refractory autoimmune mucocutaneous disease
Patricia P L Ng1, Adrienne Rencic, H Carlos Nousari
1Department of Dermatology, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
Journal of Cutaneous Medicine and Surgery
|August 28, 2002
Abstract
Background And Objective:
We report on a 42-year-old man with Stage IIA non-Hodgkin's lymphoma who developed a severe mucocutaneous blistering eruption. His diagnosis, paraneoplastic pemphigus, was based on clinical, histological, and immunofluorescence findings and confirmed by immunoprecipitation. Despite maximal therapy with plasmapheresis, corticosteroids, and mycophenolate mofetil and the subsequent addition of cyclophosphamide and cyclosporine, the condition was fatal.
Conclusion:
This case illustrates the refractory nature of this disease and the inadequacy of existing therapies.