Background: Systemic corticosteroids, with or without the addition of immunosuppressive adjuvant agents, are frequently used in treating patients with pemphigus vulgaris (PV). The severe, catastrophic, and potentially fatal side effects of these agents highlight the need for the development of safe alternatives for PV therapy. Intravenous immunoglobulin (IVIG) therapy has recently been reported to be effective in the treatment of PV. Objective: Our purpose was to do a retrospective analysis of the available literature on the use of IVIG in the treatment of PV. We also wished to determine whether the cumulative evidence permits making preliminary conclusions regarding the potential role of IVIG in the overall management of PV. Methods: A review of the English-language, peer-reviewed literature was conducted for reports on IVIG use in treatment of PV. The available information on 21 patients was used to assess different dimensions of clinical efficacy. Results: A minimum dose of 2 g/kg per cycle given at regular monthly intervals for a minimum of 3 cycles seems be effective in inducing a rapid clinical remission in patients with severe, recalcitrant PV. However, this should not be perceived as a “standard” dose. Tapering and eventual discontinuation of other agents were possible in many patients. Long-term follow-up was not provided to examine the influence of IVIG on the clinical course of disease, its efficacy as monotherapy, and the benefit of using it as maintenance therapy to keep the patient in prolonged clinical remission. In 4 of the 21 patients (19%), use of IVIG was of no clinical benefit. This failure of efficacy was primarily due to inadequate use. IVIG demonstrated beneficial effect in 17 of 21 patients (81%). Conclusion: IVIG may be a safe and effective agent in the management of severe, recalcitrant PV. Multicenter controlled studies, using different dose regimens, with lengthy follow-up periods are necessary to clearly define the emerging beneficial role of IVIG. (J Am Acad Dermatol 2000;43:1049-57.)