Ahmed A R, Hombal S
Department of Medicine, University of California at Los Angeles School of Medicine.
J Am Acad Dermatol. 1987 Sep;17(3):437-42. doi: 10.1016/s0190-9622(87)70226-6.
Four patients with pemphigus vulgaris are presented in which diagnosis was confirmed histologically and immunopathologically. Although these patients responded to high-dose prednisone therapy during the initial stages of acute disease, the addition of azathioprine failed to allow lower steroid doses and did not result in prolonged, complete remission. Indeed, the disease was exacerbated during azathioprine therapy, and significant side effects from prolonged high-dose steroid therapy were observed. Both clinical and serologic remission resulted from the addition of cyclophosphamide and dapsone to prednisone therapy. Thus, when azathioprine fails to produce remission or a steroid-sparing effect, cyclophosphamide may be an effective alternative. During a prolonged follow-up period, no recurrences of pemphigus have been observed, and no significant side effects of cyclophosphamide (Cytoxan) have been encountered. The addition of dapsone produced enhanced anti-inflammatory effects without increasing the existing or potential side effects of steroid therapy. Dapsone was easily withdrawn at the onset of remission. Thus the anti-inflammatory effect of dapsone may prove valuable in patients for whom steroids are contraindicated, who develop significant side effects during long-term steroid therapy, or for whom increases of dose threaten to enhance the possibility of catastrophic side effects.
本文报告了4例寻常型天疱疮患者，其诊断经组织学和免疫病理学证实。尽管这些患者在急性疾病初期对高剂量泼尼松治疗有反应，但加用硫唑嘌呤未能使激素剂量降低，也未导致长期完全缓解。事实上，在硫唑嘌呤治疗期间病情加重，且观察到长期高剂量激素治疗有明显的副作用。泼尼松治疗加用环磷酰胺和氨苯砜后，临床和血清学均获缓解。因此，当硫唑嘌呤不能产生缓解或激素节约效应时，环磷酰胺可能是一种有效的替代药物。在长期随访期间，未观察到天疱疮复发，也未遇到环磷酰胺（癌得星）的明显副作用。加用氨苯砜可增强抗炎作用，而不增加激素治疗现有的或潜在的副作用。在缓解开始时可轻易停用氨苯砜。因此，氨苯砜的抗炎作用可能对那些禁忌使用激素、在长期激素治疗期间出现明显副作用或增加剂量可能增加灾难性副作用可能性的患者有价值。
