Evidence-based treatments for pemphigus vulgaris, pemphigus foliaceus, and bullous pemphigoid: a systematic 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.