A 54-year-old woman with severe bullous pemphigoid (BP) associated with insulin dependent diabetes mellitus (DM), who showed unresponsiveness to conventional therapy with corticosteroids in combination of either cyclosporme or dapsone, was successfully treated with plasmapheresis followed each time by 3 successive days of pulse therapy of cyclophosphamide (500mg, intravenously). After six times of plasmapheresis, anti-basement membrane zone (BMZ) antibody titer decreased from 1:1280 to 1:40 and no new lesions developed at all. In severe refractory BP patients with uncontrolled DM, plasmapheresis is one of the valuable treatment modalities for a short period and the need for corticosteroids thus avoiding corticosteroid induced side effects.