Abstract
BACKGROUND: A range of interventions has been described for the treatment of pemphigus; however, the optimal therapeutic strategy has not been established. OBJECTIVE: We sought to evaluate the safety and efficacy of interventions for pemphigus vulgaris and pemphigus foliaceus. METHODS: We undertook a systematic review and meta-analysis according to the methodology of the Cochrane Collaboration. We selected randomized controlled trials including participants with the diagnosis of pemphigus vulgaris or pemphigus foliaceus confirmed with clinical, histopathological, and immunofluorescence criteria. All interventions were considered. Primary outcomes studied were remission and mortality. Secondary outcomes included disease control, relapse, pemphigus severity score, time to disease control, cumulative glucocorticoid dose, serum antibody titers, adverse events, and quality of life. RESULTS: Eleven studies with a total of 404 participants were identified. Interventions assessed included prednisolone dose regimen, pulsed dexamethasone, azathioprine, cyclophosphamide, cyclosporine, dapsone, mycophenolate, plasma exchange, topical epidermal growth factor, and traditional Chinese medicine. We found some interventions to be superior for certain outcomes, although we were unable to conclude which treatments are superior overall. LIMITATIONS: Many interventions for pemphigus have not been evaluated in controlled trials. All studies were insufficiently powered to establish definitive results. CONCLUSIONS: There is inadequate evidence available at present to ascertain the optimal therapy for pemphigus vulgaris and pemphigus foliaceus. Further randomized controlled trials are required.