Treatment options for pemphigus foliaceus.

Pemphigus foliaceus belongs to a group of rare autoimmune blistering mucocutaneous conditions. Systemic corticosteroids have markedly reduced the mortality from the disease but its utility is restricted by adverse events. This is further complicated by the fact that steroid-sparing agents are also associated with serious adverse events and there are only few randomized controlled trials demonstrating a beneficial response from the use of these agents. Azathioprine and mycophenolate mofetil appear to be the most feasible first line adjunctive agents in terms of inducing and maintaining remission and harboring a comparatively favourable side effect profile. An enhanced understanding of the pathogenesis of pemphigus has resulted in the implementation of a number of novel therapies including biological agents, intravenous immunoglobulin, and extracorporeal treatment modalities. These therapies have also been mainly studied through case series reports, are expensive and/or difficult to access in some centres, and are associated with a number of deleterious side effects. Rituximab, the anti-CD20 chimeric monoclonal antibody, is currently emerging as the therapy of choice in severe refractory disease. Further studies on the effects and safety profiles of more specific agents, such as peptide immunotherapy and the targeting of intracellular signalling molecules involved in the pathogenesis of pemphigus are needed.