title: Interventions for pemphigus vulgaris and pemphigus foliaceus.
abstract: BACKGROUND
  A range of interventions have been described for treatment of pemphigus, however the optimal therapeutic strategy has not been established.
  
  
  OBJECTIVE
  To assess the efficacy and safety of all interventions used in the management of pemphigus vulgaris and pemphigus foliaceus.
  
  
  METHODS
  We searched the Cochrane Skin Group Specialised Register (October 2008), The Cochrane Central Register of Controlled Trials (The Cochrane Library Issue 4, 2008), MEDLINE (2003 to October 2008), EMBASE (2005 to October 2008), LILACS (1981 to October 2008), Ongoing Trials Registers, reference lists of articles, conference proceedings from international pemphigus meetings and contacted experts in the field.
  
  
  METHODS
  Randomised controlled trials of any intervention in pemphigus vulgaris or pemphigus foliaceus.
  
  
  METHODS
  Two authors independently assessed quality and extracted data from studies. All investigators were contacted for further information. Adverse events were identified from included studies.
  
  
  RESULTS
  Eleven studies with a total of 404 participants (337 pemphigus vulgaris, 27 pemphigus foliaceus and 40 not specified ) were identified. The quality of included studies was not high, the majority of studies did not report allocation concealment, and power was limited by very small sample sizes. Interventions assessed included prednisolone dose regimen, pulsed dexamethasone, azathioprine, cyclophosphamide, cyclosporine, dapsone, mycophenolate, plasma exchange, topical epidermal growth factor and traditional Chinese medicine. Ten studies included participants with newly diagnosed or newly active recurrent disease, and one trial included participants in maintenance phase.There was sufficient data for 4 meta-analyses, each pooling results of two studies only. For the majority of interventions, results were inconclusive. We found some interventions to be superior for certain outcomes, although we were unable to conclude which treatments are superior overall. Mycophenolate was more effective in achieving disease control than azathioprine (1 study; n=40; RR 0.72; 95% CI 0.52 to 0.99, NNT 3.7). There was evidence of a steroid-sparing benefit of azathioprine (1 study; n=57; MWD -3919 mg prednisolone; 95% CI -6712 to -1126) and cyclophosphamide (1 study; n=54; MWD -3355 mg prednisolone; 95% CI -6144 to -566) compared to glucocorticoids alone. Topical epidermal growth factor decreased time to control (1 study; n=20; HR 2.35; 95% CI 1.62 to 3.41).
  
  
  CONCLUSIONS
  There is inadequate information available at present to ascertain the optimal therapy for pemphigus vulgaris or pemphigus foliaceus. Further research is required, especially to assess the optimal glucocorticoid dose, the role of adjuvant immunosuppressive medications, and long-term adverse events to improve harm:benefit analyses.
authors.affiliation: Department of Dermatology , St George Hospital, Gray St, Kogarah, Sydney, NSW, Australia, 2217.
authors.forename: Linda K
authors.initials: LK
authors.lastname: Martin
authors.forename: Victoria
authors.initials: V
authors.lastname: Werth
authors.forename: Elmer
authors.initials: E
authors.lastname: Villanueva
authors.forename: Janet
authors.initials: J
authors.lastname: Segall
authors.forename: Dedee F
authors.initials: DF
authors.lastname: Murrell
mesh_terms: D005938:Glucocorticoids / Q000627:therapeutic use*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D010392:Pemphigus / Q000145:classification / Q000188:drug therapy*; D016032:Randomized Controlled Trials as Topic
chemical_list: D005938:Glucocorticoids; D007166:Immunosuppressive Agents