Treatment of bullous pemphigoid and pemphigus. The French experience, 2009 update

There have been several recent advances in the management of auto-immune blistering diseases. The aim of this review was to provide an update, based on ''expert'' interpretation of published evidences on the treatments of pemphigoid and pemphigus. A literature search (Pubmed) of all randomized controlled trials that had not been included in two reviews published by the Cochrane Skin Group in 2005 on pemphigoid and 2009 on pemphigus have been performed. Recent series of pemphigus cases treated with anti-CD20 antibodies have also been reviewed. Concerning pemphigoid there is good evidence that high dose topical clobetasol propionate should be the first line treatment, and that further studies should focus on the finding of simple and well tolerated maintenance therapy. For pemphigus, after decades of inconclusive trials, there is now some evidence of a moderate efficacy of azathioprine, cyclophosphamide, mycophenolate in addition to corticosteroids and may be of intravenous immunoglobulins. The practical impact of such evidence is strongly challenged by the possible superiority of anti-CD20 monoclonal antibodies that may emerge as the most efficient treatment of pemphigus. Because of concerns on long-term safety, multicenter trials of these ''biological'' treatments should be undertaken urgently.