Burruss J B, Farmer E R, Callen J P
Division of Dermatology, University of Louisville School of Medicine, KY, USA.
J Am Acad Dermatol. 1996 Nov;35(5 Pt 1):720-4. doi: 10.1016/s0190-9622(96)90727-6.
Pyoderma gangrenosum (PG) may fail to respond to corticosteroids. Immunosuppressive and cytotoxic agents are useful in patients with recalcitrant disease. We describe our experiences with chlorambucil for PG.
Our purpose was to evaluate the effectiveness of oral chlorambucil in patients with PG recalcitrant to treatment with prednisone, immunosuppressive therapy, or both.
Six patients with recalcitrant PG were given oral chlorambucil 2 to 4 mg/day. Four patients were treated with a combination of prednisone and chlorambucil, and two received chlorambucil alone. Response was based on (1) a diminution in the size of the ulcers, or their complete healing, or (2) a decrease in the dose of corticosteroid therapy.
Beneficial effects were noted within 6 to 8 weeks in all six patients, and corticosteroids were eventually discontinued in all patients. Currently only two patients are still receiving chlorambucil; the other four stopped chlorambucil after 6 to 24 months of treatment. Their disease has remained in remission for 4 to 9 years. Relapse of disease occurred within 1 to 4 months after stopping therapy in one of the two remaining patients or reducing the dose in the other. In both patients, the disease is again responding to treatment. Minimal chlorambucil toxicity has been noted, consisting of leukopenia in one patient.
Our findings suggest that chlorambucil is an effective corticosteroid-sparing agent for the control of PG.
坏疽性脓皮病(PG)可能对皮质类固醇无反应。免疫抑制剂和细胞毒性药物对难治性疾病患者有用。我们描述了我们使用苯丁酸氮芥治疗PG的经验。
我们的目的是评估口服苯丁酸氮芥对泼尼松、免疫抑制治疗或两者治疗均无效的PG患者的有效性。
6例难治性PG患者口服苯丁酸氮芥2至4mg/天。4例患者接受泼尼松和苯丁酸氮芥联合治疗,2例仅接受苯丁酸氮芥治疗。疗效基于(1)溃疡大小减小或完全愈合,或(2)皮质类固醇治疗剂量减少。
所有6例患者在6至8周内均出现有益效果,所有患者最终均停用皮质类固醇。目前只有2例患者仍在接受苯丁酸氮芥治疗;其他4例在治疗6至24个月后停止使用苯丁酸氮芥。他们的疾病已缓解4至9年。在剩下的2例患者中,1例在停药后1至4个月内疾病复发,另1例在减药后复发。在这2例患者中,疾病再次对治疗有反应。已注意到苯丁酸氮芥的毒性极小,仅1例患者出现白细胞减少。
我们的研究结果表明,苯丁酸氮芥是一种有效的皮质类固醇替代药物,可用于控制PG。