The therapy of pemphigus is one of the miracles of modern medicine, having transformed an almost invariably fatal disease into one whose mortality is now approximately 5%. Nonetheless, major challenges remain in further decreasing mortality and reducing the side effects of the potent medications used to treat this disease. The cornerstone of treatment remains the use of systemic steroids. The role of adjuvants, such as immunosuppressive and anti-inflammatory drugs, as ''steroid-sparing'' agents, remains to be defined, as there are little randomized trial data that provides objective evidence of their effectiveness. More recently, biologicals, such as IVIg and rituximab, have been introduced; the first to speed the control of active disease unresponsive to conventional treatment and the second to increase the proportion of patients in whom a complete clinical remission can be induced.