Department of Dermatology, Venereology, and Allergology, University Medical Center Göttingen, Germany, Robert Koch Str. 40, D-37075 Göttingen, Germany.
Department of Dermatology, Venereology, and Allergology, University Medical Center Göttingen, Germany, Robert Koch Str. 40, D-37075 Göttingen, Germany, Lower Saxony Institute of Occupational Dermatology, University Medical Center Göttingen, Germany.
Eur J Dermatol. 2021 Dec 1;31(6):822-829. doi: 10.1684/ejd.2021.4173.
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.
We present three new patients with generalized morphea treated with intravenous immunoglobulins as well as a review of the literature.
MATERIALS & 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.
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.
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.
泛发性硬斑病和嗜酸性筋膜炎是两种难以治疗的炎症性和硬化性皮肤病。据报道，静脉注射免疫球蛋白可能具有免疫调节和抗纤维化特性，对少数此类疾病有效。
我们报告三例新的泛发性硬斑病患者，这些患者接受了静脉注射免疫球蛋白治疗，并对文献进行了回顾。
描述了三例住院的泛发性硬斑病患者（年龄分别为 66 岁、65 岁和 67 岁，均为男性），他们均为首次接受治疗。
这三例患者接受了静脉注射免疫球蛋白（1.5-2 g/kg 体重，连续 3-4 天，每 4 周一次）治疗。所有患者均联合了皮质类固醇脉冲治疗，两例患者联合使用了甲氨蝶呤，一例患者联合使用了霉酚酸酯。治疗开始后 1-5 个月，所有患者的皮肤硬化均明显且稳定改善。未观察到不良反应。迄今为止，有 12 篇文献报道了 16 例泛发性硬斑病或嗜酸性筋膜炎患者接受了静脉注射免疫球蛋白治疗。大多数患者（9/16）治疗效果显著，且具有良好的风险特征。
我们的病例进一步证明，静脉注射免疫球蛋白联合糖皮质激素和传统免疫抑制剂治疗硬斑病是一种安全有效的治疗方法。在未来高质量的研究中，验证这些结果似乎是合适的。
