M E Fleischli1, R H Valek, A G Pandya. 1. Department of Dermatology, University of Texas Southwestern Medical Center, Dallas 75235-9069, USA.
Abstract
BACKGROUND: Adjuvant therapy is commonly used in pemphigus to mitigate the high morbidity and mortality associated with the use of corticosteroids and improve disease control. However, these adjuvant agents are not without adverse effects of their own, including an increased risk of malignancy with the use of oral immunosuppressives. Intravenous pulse cyclophosphamide, which may be more efficacious and less toxic than oral immunosuppressives, has been used successfully in the treatment of pemphigus. OBJECTIVE: To review 9 patients with severe or previously recalcitrant pemphigus who were treated with intravenous pulse cyclophosphamide therapy. RESULTS: Six of the 9 patients responded to therapy, with 2 patients achieving remission from skin lesions. Five patients were able to decrease their daily dose of prednisone, and 1 was able to discontinue the use of prednisone completely. Most patients experienced minimal or no adverse effects. CONCLUSIONS: Intravenous pulse cyclophosphamide may be an alternative treatment option in patients with pemphigus recalcitrant to standard therapy. The decreased cumulative dose of cyclophosphamide observed with monthly pulse doses may reduce the incidence of secondary malignancies when compared with continuous oral therapy. Controlled trials are needed to further evaluate the efficacy of this mode of therapy.
BACKGROUND: Adjuvant therapy is commonly used in pemphigus to mitigate the high morbidity and mortality associated with the use of corticosteroids and improve disease control. However, these adjuvant agents are not without adverse effects of their own, including an increased risk of malignancy with the use of oral immunosuppressives. Intravenous pulse cyclophosphamide, which may be more efficacious and less toxic than oral immunosuppressives, has been used successfully in the treatment of pemphigus. OBJECTIVE: To review 9 patients with severe or previously recalcitrant pemphigus who were treated with intravenous pulse cyclophosphamide therapy. RESULTS: Six of the 9 patients responded to therapy, with 2 patients achieving remission from skin lesions. Five patients were able to decrease their daily dose of prednisone, and 1 was able to discontinue the use of prednisone completely. Most patients experienced minimal or no adverse effects. CONCLUSIONS: Intravenous pulse cyclophosphamide may be an alternative treatment option in patients with pemphigus recalcitrant to standard therapy. The decreased cumulative dose of cyclophosphamide observed with monthly pulse doses may reduce the incidence of secondary malignancies when compared with continuous oral therapy. Controlled trials are needed to further evaluate the efficacy of this mode of therapy.