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Giornale Italiano di Dermatologia e Venereologia 2007 August;142(4):391-408


language: English

Drug therapy of pemphigus vulgaris

Ahmed A. R.

Center for Blistering Diseases New England Baptist Hospital Boston, MA, USA


Pemphigus vulgaris (PV) was and continues to remain a potentially fatal, autoimmune, mucocutaneous blistering disease. It most frequently affects the oral cavity and skin or both. Other mucous membranes of eye, nose, pharynx, larynx, esophagus, trachea, vagina, penis, anal canal, and the nails can be affected. It is characterized by an intraepidermal suprabasilar cleft, with in vivo deposition of IgG on perilesional tissue and a circulating autoantibody to keratinocyte cell surface molecules. Since their initial use, systemic corticosteroids have remained the mainstay of treatment. New agents have been used as they became available. Amongst the immunosuppressive agents available, the most commonly used are azathioprine, mycophenolate mofetil in combination with prednisone. Similar results with cyclophosphamide when used in a dexamethasone - cyclophosphamide pulse therapy protocol are observed. Immunoablative therapy without stem cell rescue has been abandoned. Plasmapheresis has limited value, as does extracorporeal photophoresis. Immunoadsorption shows promise but requires additional research to determine the optimal condition for the variables associated with the procedure. On the horizon the biologic agents show the most promise. Intravenous immunoglobulin (IVIg) is safe, produces long term remissions, has few side effects, but requires the presence of an infrastructure for delivery. It is slow to act. Not all patients have a complete response. Studies to attempt to convert poor responders to high responders are underway but not completed. A monoclonal antibody (rituximab) to CD20 present on pathogenic autoantibody producing cells has the potential to produce long term remissions. Used according to a lymphoma protocol, it can result in severe infection and death, besides a reasonable rate of recurrence. Its use with IVIg on a unique protocol may produce better results and less fatal infections. There is a renewed interest in the treatment of PV. Many investigators believe that it is a good model for autoimmunity. Lessons learned from treating pemphigus may be applied to other autoimmune diseases.

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