A thirty-one-year-old man consulted our clinic with widespread erythema, blisters and erosions on his body surface. These skin lesions rapidly deteriorated until they extended over 70% of his skin surface area and the patient showed a high serum level of anti-desmoglein 1 antibody (880 index). Based on the diagnosis of severe pemphigus foliaceus, we started rapid treatment with combination therapy consisting of oral prednisolone (70 mg/day at first dosage), double-filtration plasmapheresis (DFPP) and high-dose intravenous immunoglobulin (IVIG), which successfully achieved remission. Therefore, this combination therapy is considered useful in the treatment of severe pemphigus foliaceus in order to reduce corticosteroid dosage smoothly without disease relapse and to shorten the duration of admission.