Abstract
We reported a case of pemphigus vulgaris (PV) successfully treated with double filtration plasmapheresis (DFPP). A 50-year-old man developed blisters and erosions on his face, oral mucosa, scalp and trunk from June of 2000. Clinical, histological and immunofluorescence findings confirmed the diagnosis of PV. He was initially treated with predonisolone (PSL) 60mg/day, and his cutaneous and mucosal lesions gradually improved. However, when the dose of PSL was tapered, his cutaneous and oral lesions exacerbated. After three cycles of exacerbation of his lesion, we chose DFPP therapy. After DFPP, his clinical symptoms cleared promptly. The titer of anti-desmoglein(Dsg)-1 autoantibodies by ELISA decreased from 180 to 25 and that of anti-Dsg-3 autoantibodies from 3,780 to 180. We consider that DFPP is very useful for the treatment of PV. In the present case, there was a good correlation between the titer of anti-Dsg autoantibodies and clinical symptoms. When the titer of anti-Dsg-1autoantibodies exceeded 140 and that of anti-Dsg-3 autoantibodies exceeded 2,400, the cutaneous and mucosal lesions appeared. It was possible to predict the recurrence of symptoms from the titer of anti-Dsg autoantibodies and to plan the therapy in advance.
