Pemphigus Vulgaris Treatment
Therapy is aimed at reducing antibody synthesis. Unlike disorders such as bullous pemphigoid or dermatitis herpetiformis where there is a large inflammatory component in addition to the immune component, anti-inflammatory drugs have no known benefit in the treatment of pemphigus vulgaris. It is generally agreed that patients with only limited initial disease need as much intensive therapy to induce a remission as patients with extensive involvement.
Prednisone is the standard initial therapy unless contraindicated. Most patients obtain a complete or almost complete clinical remission within 8 to 10 weeks and then the dose can be tapered gradually. Low doses are then maintained for several years. Other immunosuppressive drugs such as azathioprine or cyclophosphamide may be added if prednisone fails to induce a remission or if the patient develops serious corticosteroid complications. Mycophenolate mofetil, methotrexate, and cyclosporin are added to prednisone as adjuvant therapies less frequently. Other treatments that have been utilized include intravenous pulse methylprednisolone, intravenous pulse cyclophosphamide, adjuvant intravenous gammaglobulin, immunoablative intravenous cyclophasphamide without stem cell rescue, intramuscular gold, and extracorporeal photochemotherapy.
Death formerly occurred in almost all cases. However, with the development of immunosuppressive therapy, the mortality rate of PV is now approximately 6%. Infection is often the cause of death and immunosuppressive therapy is typically a contributing factor. Most deaths occur within the first few years of disease onset. If the patient survives greater than five years after the inception of disease, the patient’s outcome is usually excellent.