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Indian Journal of Dermatology,... 2011Pemphigus, bullous pemphigoid, and epidermolysis bullosa acquisita are autoimmune diseases of skin associated with considerable morbidity and sometimes mortality. There... (Review)
Review
BACKGROUND
Pemphigus, bullous pemphigoid, and epidermolysis bullosa acquisita are autoimmune diseases of skin associated with considerable morbidity and sometimes mortality. There is no cure for these diseases.
AIMS
To summarize evidence-based treatments for these diseases by performing a systematic review.
METHODS
The research protocol included the following steps: identification of databases to be searched, defining search strategy, searching the databases for references, first-stage screening of the abstracts, second-stage screening of full texts of articles identified after the first-stage screening, data extraction from the identified articles after second-stage screening, quality appraisal of the studies using the Delphi list, and summarizing the findings.
RESULTS
No randomized controlled trials of interventions in pemphigus vegetans, pemphigus erythematosus, and epidermolysis bullosa acquisita could be found. After the second-stage screening, 12 randomized controlled trials were analyzed, which included patients with pemphigus vulgaris or pemphigus vulgaris and pemphigus foliaceus, and 7 which included patients with bullous pemphigoid.
CONCLUSIONS
Number of high-quality randomized controlled trials conducted on pemphigus and bullous pemphigoid is small. Oral corticosteroid along with a steroid-sparing agent appears to be the most effective treatment for pemphigus. Azathioprine may be most effective as a steroid-sparing agent. Topical corticosteroid therapy (as studied) is effective for bullous pemphigoid and appears to be superior to oral corticosteroid for extensive disease. Some suggestions about future research are made.
Topics: Animals; Evidence-Based Medicine; Humans; Immunosuppressive Agents; Pemphigoid, Bullous; Pemphigus; Randomized Controlled Trials as Topic
PubMed: 21727693
DOI: 10.4103/0378-6323.82400 -
Journal of the American Academy of... May 2011A range of interventions has been described for the treatment of pemphigus; however, the optimal therapeutic strategy has not been established. (Meta-Analysis)
Meta-Analysis Review
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.
Topics: Azathioprine; Cyclophosphamide; Epidermal Growth Factor; Glucocorticoids; Humans; Immunosuppressive Agents; Mycophenolic Acid; Pemphigus; Randomized Controlled Trials as Topic; Remission Induction; Treatment Outcome
PubMed: 21353333
DOI: 10.1016/j.jaad.2010.04.039