Taïeb A, Klene C, Maleville J
Ann Dermatol Venereol. 1986;113(12):1223-9.
Optimal management of the patient with generalized bullous pemphigoid (BP) should both control the disease rapidly to avoid fluid losses and superinfection, and have minimal side-effects. Although their initial dosage is subject to some controversy, corticosteroids (CS) provide currently the basis of therapy. Immunosuppressants have been advocated to decrease CS dosages more rapidly and to reduce potential hazards of long-term systemic steroid therapy, because of their sparing effect on cumulated CS doses. They have a delayed activity ranging from three to six weeks after beginning of therapy. Chloraminophene has been used in most previous studies. The aim of this study was to assess retrospectively a combined CS-cyclophosphamide (CP) regimen. Ten patients with generalized BP were treated. In nine, dexamethasone sodium phosphate was given intravenously (mean: 1.56 mg/kg/day in prednisone equivalents; range: 1 to 1.90 mg/kg/day) at onset of treatment. Prednisolone was given orally when complete control--absence of any new lesion--was achieved. CP was given d'emblée at a daily dosage of 100 mg for at least 6 months when feasible (mean: 4 months; range 9 days to 15 months). Patients characteristics are summarized in table I. Results are shown in tables II and III. In brief, three deaths occurred following major side-effects of the drugs during the first three months of treatment. Further non lethal untoward effects included bone marrow toxicity due to CP and septicemia (4 patients). At the end of follow-up, 5 patients were in remission without treatment for 22 +/- 6 months and one was controlled with prednisolone alone.(ABSTRACT TRUNCATED AT 250 WORDS)
泛发性大疱性类天疱疮(BP)患者的最佳管理应既能迅速控制病情以避免液体流失和继发感染,又能使副作用最小化。尽管其初始剂量存在一些争议,但目前糖皮质激素(CS)是治疗的基础。免疫抑制剂因其对累积CS剂量的节省作用,被提倡用于更快降低CS剂量并减少长期全身使用类固醇治疗的潜在危害。它们在治疗开始后三到六周才会发挥延迟作用。在大多数先前的研究中都使用了氯苯吩嗪。本研究的目的是回顾性评估CS-环磷酰胺(CP)联合治疗方案。对10例泛发性BP患者进行了治疗。9例患者在治疗开始时静脉注射地塞米松磷酸钠(以泼尼松等效剂量计,平均:1.56 mg/kg/天;范围:1至1.90 mg/kg/天)。当实现完全控制(即无任何新皮损)时,改为口服泼尼松龙。可行时,CP从一开始就以每日100 mg的剂量给药至少6个月(平均:4个月;范围9天至15个月)。患者特征总结于表I。结果见表II和III。简而言之,在治疗的前三个月,有3例患者因药物的严重副作用死亡。其他非致命的不良影响包括CP引起的骨髓毒性和败血症(4例患者)。随访结束时,5例患者未经治疗缓解了22±6个月,1例仅用泼尼松龙控制。(摘要截短于250字)