Abstract：
BACKGROUND: Generalized morphea and eosinophilic fasciitis are difficult-to-treat inflammatory and sclerosing skin diseases. Few cases have been reported in which intravenous immunoglobulins were of benefit, possibly owing to their immunomodulatory and antifibrotic properties.
OBJECTIVE: We present three new patients with generalized morphea treated with intravenous immunoglobulins as well as a review of the literature.
METHODS: Three hospitalized patients (two men, age 66 and 65 years, respectively, and a 67-year-old woman) with generalized morphea who received therapy for the first time are described.
RESULTS: The three patients were treated with intravenous immunoglobulins (1.5-2 g/kg body weight over three to four consecutive days every four weeks). This was combined with corticosteroid pulse therapy in all patients, methotrexate in two patients and mycophenolate mofetil in one patient, respectively. Marked and steady improvement of skin sclerosis was evident in all patients, one to five months after treatment initiation. No adverse events were observed. To date, there are 12 reports of 16 patients with generalized morphea or eosinophilic fasciitis treated with intravenous immunoglobulins. The treatment was highly effective in the majority of patients (9/16) and yielded a favourable risk profile.
CONCLUSIONS: Our cases add to the hitherto limited evidence that the administration of intravenous immunoglobulins in combination with glucocorticoids and conventional immunosuppressive agents is a safe and effective therapy against morphea. It seems appropriate to verify these results in future high-quality studies.
OBJECTIVE: We present three new patients with generalized morphea treated with intravenous immunoglobulins as well as a review of the literature.
METHODS: Three hospitalized patients (two men, age 66 and 65 years, respectively, and a 67-year-old woman) with generalized morphea who received therapy for the first time are described.
RESULTS: The three patients were treated with intravenous immunoglobulins (1.5-2 g/kg body weight over three to four consecutive days every four weeks). This was combined with corticosteroid pulse therapy in all patients, methotrexate in two patients and mycophenolate mofetil in one patient, respectively. Marked and steady improvement of skin sclerosis was evident in all patients, one to five months after treatment initiation. No adverse events were observed. To date, there are 12 reports of 16 patients with generalized morphea or eosinophilic fasciitis treated with intravenous immunoglobulins. The treatment was highly effective in the majority of patients (9/16) and yielded a favourable risk profile.
CONCLUSIONS: Our cases add to the hitherto limited evidence that the administration of intravenous immunoglobulins in combination with glucocorticoids and conventional immunosuppressive agents is a safe and effective therapy against morphea. It seems appropriate to verify these results in future high-quality studies.
摘要：
背景：全身性硬皮和嗜酸性筋膜炎是难以治疗的炎症性和硬化性皮肤病。很少有报道说静脉注射免疫球蛋白是有益的，可能是由于它们的免疫调节和抗纤维化特性。
目的：我们介绍了3例用静脉注射免疫球蛋白治疗的广泛性硬叶患者，并对文献进行了综述。
方法：三名住院患者（两名男性，年龄66和65岁，分别,描述了一名67岁的女性）首次接受治疗的泛发性疣。
结果：三名患者接受了静脉免疫球蛋白（每四周连续三到四天，1.5-2g/kg体重）治疗。所有患者都接受了皮质类固醇脉冲治疗，甲氨蝶呤治疗2例，霉酚酸酯治疗1例，分别。在所有患者中皮肤硬化的显著和稳定的改善是明显的,治疗开始后一到五个月。没有观察到不良事件。迄今为止,有12例报告，其中16例患者接受静脉注射免疫球蛋白治疗，患有广泛性硬叶或嗜酸性筋膜炎。该治疗在大多数患者中非常有效（9/16），并产生了良好的风险状况。
结论：我们的病例增加了迄今为止有限的证据，即静脉内免疫球蛋白与糖皮质激素和常规免疫抑制剂联合给药是一种安全有效的治疗硬伤的方法。在未来的高质量研究中验证这些结果似乎是合适的。
目的：我们介绍了3例用静脉注射免疫球蛋白治疗的广泛性硬叶患者，并对文献进行了综述。
方法：三名住院患者（两名男性，年龄66和65岁，分别,描述了一名67岁的女性）首次接受治疗的泛发性疣。
结果：三名患者接受了静脉免疫球蛋白（每四周连续三到四天，1.5-2g/kg体重）治疗。所有患者都接受了皮质类固醇脉冲治疗，甲氨蝶呤治疗2例，霉酚酸酯治疗1例，分别。在所有患者中皮肤硬化的显著和稳定的改善是明显的,治疗开始后一到五个月。没有观察到不良事件。迄今为止,有12例报告，其中16例患者接受静脉注射免疫球蛋白治疗，患有广泛性硬叶或嗜酸性筋膜炎。该治疗在大多数患者中非常有效（9/16），并产生了良好的风险状况。
结论：我们的病例增加了迄今为止有限的证据，即静脉内免疫球蛋白与糖皮质激素和常规免疫抑制剂联合给药是一种安全有效的治疗硬伤的方法。在未来的高质量研究中验证这些结果似乎是合适的。
