title: 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.
  
  
  OBJECTIVE
  The objective of this systematic review is to assess the literature on the efficacy and safety of interventions for the treatment of pemphigus vulgaris.
  
  
  METHODS
  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.
  
  
  METHODS
  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.
  
  
  METHODS
  Altogether 18 RCTs were identified including 16 distinct interventions.
  
  
  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.
  
  
  CONCLUSIONS
  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
  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.
authors: Department of Dermatology, St George Hospital, Gray St, Sydney, NSW, 2217, Australia.
authors: Cathy Y
authors: CY
authors: Zhao
authors: Dedee F
authors: DF
authors: Murrell
mesh_terms: D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000008:administration & dosage / Q000009:adverse effects / Q000627:therapeutic use*; D004305:Dose-Response Relationship, Drug; D005938:Glucocorticoids / Q000008:administration & dosage / Q000009:adverse effects / Q000627:therapeutic use*; D006801:Humans; D007155:Immunologic Factors / Q000008:administration & dosage / Q000009:adverse effects / Q000627:therapeutic use*; D010392:Pemphigus / Q000188:drug therapy* / Q000276:immunology; D016032:Randomized Controlled Trials as Topic; D016896:Treatment Outcome
chemical_list: D000894:Anti-Inflammatory Agents, Non-Steroidal; D005938:Glucocorticoids; D007155:Immunologic Factors
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