Pemphigus vulgaris: an evidence-based treatment update

Abstract

Background: While a variety of intervention options have been described for pemphigus vulgaris, the optimal treatment strategy has not been established.

Objectives: The objective of this systematic review is to assess the literature on the efficacy and safety of interventions for the treatment of pemphigus vulgaris.

Data sources: Five electronic databases were searched, including The Cochrane Skin Group's Specialized Register, The Cochrane Central Register of Controlled Trials (CENTRAL), EMBASE, MEDLINE and Latin American and Caribbean Health science Information database (LILACS). Five trial registers as well as reference lists of included RCTs were also searched.

Study eligibility criteria: Any published randomised controlled trial (RCT) on intervention for pemphigus vulgaris was included, provided the diagnosis of pemphigus vulgaris was confirmed with appropriate clinical features, histopathology and immunofluorescence studies. Studies which included forms of pemphigus other than pemphigus vulgaris were excluded.

Interventions: Altogether 18 RCTs were identified including 16 distinct interventions.

Study appraisal and synthesis methods: Included studies were assessed for patient selection, methods of randomisation, blinding, follow-up and selective reporting.

Results: Current evidence is incomplete and inconclusive. The interventions which appear promising, but will require further evaluation include adjuvant mycophenolate mofetil (MMF), azathioprine, intravenous immunoglobulins (IVIG), sulfasalazine and pentoxifylline, infliximab, epidermal growth factor and pimecrolimus. Interventions with inconclusive evidence include high (120-180 mg) versus low (45-60 mg) prednisone dosage, pulsed dexamethasone, cyclophosphamide, dexamethasone-cyclophosphamide pulse therapy (DCP), cyclosporine, dapsone, etanercept, acyclovir and tacrolimus.

Limitations: Our review is limited by the small number of high-quality RCTs and variety of outcome measures, precluding the performing of a meta-analysis.

Conclusions and implications of key findings: The optimal treatment strategy for pemphigus vulgaris remains unclear. Higher quality RCTs are required in the future to re-evaluate many interventions and to explore other unstudied interventions.