Pemphigus vulgaris is a potentially fatal autoimmune mucocutaneous blistering disease. Conventional therapy consists of high-dose corticosteroids, immunosuppressive agents, and intravenous immune globulin. More recently newer agenst, such as Cellcept and Dapsone. Among the new approaches is Rituxan. A number of case reprots suggest that Rituxan is an effective reatment but it had not been prospectively studies.For example, a recent report of 11 pateitns treated with Rituxan and IVIG, showed that of 11 patients, 9 had rapid resolution of lesions and a clinical remission lasting 22 to 37 months (mean, 31.1). All immunosuppressive therapy, including prednisone, could be discontinued before ending rituximab treatment in all patients. Two patients were treated with rituximab only during recurrences and had sustained remissions.
Rituxan as been rapidly adopted and is now extensively used. It should be consideeds it appropriate in steroid refractory disease. There are no FDA approved therapies for this disease and Rituxan is off-label.
Harman KE, Albert S, Black MM. Guidelines for the management of pemphigus vulgaris. Br J Dermatol 2003 Nov;149(5):926-37.
Ahmed, A. Razzaque, Spigelman, Zachary, Cavacini, Lisa A., Posner, Marshall R.
Treatment of Pemphigus Vulgaris with Rituximab and Intravenous Immune Globulin
N Engl J Med 2006 355: 1772-1779
Diaz, Luis A. Rituximab and Pemphigus — A Therapeutic Advance N Engl J Med 2007 357: 605-607
Martin LK, Werth VP, Villaneuva EV, Murrell DF. A systematic review of randomized controlled trials for pemphigus vulgaris and pemphigus foliaceus. J Am Acad Dermatol 2011; 64:903.
Martin LK, Werth V, Villanueva E, et al. Interventions for pemphigus vulgaris and pemphigus foliaceus. Cochrane Database Syst Rev 2009; :CD006263.
.Mao X, Payne AS.Seeking approval: present and future therapies for pemphigus vulgaris.Curr Opin Investig Drugs. 2008 May;9(5):497-504
Eming R, Nagel A, Wolff-Franke S, et al. Rituximab exerts a dual effect in pemphigus vulgaris. J Invest Dermatol 2008; 128:2850.
Mouquet H, Musette P, Gougeon ML, et al. B-cell depletion immunotherapy in pemphigus: effects on cellular and humoral immune responses. J Invest Dermatol 2008; 128:2859.