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.

Keywords
Bullous pemphigoid, evidence-based treatment, interventions, pemphigus, review, systematic review, treatment