Division of Allergy and Immunology, Charité - Universitätsmedizin Berlin, DE-10117 Berlin, Germany.
Acta Derm Venereol. 2020 Oct 21;100(17):adv00298. doi: 10.2340/00015555-3662.
Intravenous immunoglobulins are an effective and well-tolerated treatment option for immune dermatological diseases. However, they are primarily used to treat diseases with a severe course and are mostly used off-label. Therefore, it is important to document case series on the use of intravenous immunoglobulins in rare immune dermatological diseases. We present here 32 patients who were treated with intravenous immunoglobulins in our clinic between 2015 and 2020. The indications were dermatomyositis, including amyopathic and paraneoplastic forms, as well as overlap-syndromes (n = 18), pemphigus vulgaris (n = 2), mucous membrane pemphigoid (n = 2), linear IgA dermatosis (n = 1), necrotizing vasculitis (n = 1), urticarial vasculitis (n = 1), systemic scleroderma (n = 1), Stevens-Johnson syndrome (n = 1), pyoderma gangrenosum (n = 3), and livedoid vasculopathy (n = 2). The data from this case series confirm the efficacy and tolerability of intravenous immunoglobulins as an adjuvant treatment option for rare therapy-resistant immune dermatoses.
静脉注射免疫球蛋白是治疗免疫性皮肤病的一种有效且耐受性良好的治疗选择。然而,它们主要用于治疗病情严重的疾病,并且大多是超适应证使用。因此,记录静脉注射免疫球蛋白在罕见免疫性皮肤病中的应用病例系列非常重要。我们在此介绍了 2015 年至 2020 年间在我们诊所接受静脉注射免疫球蛋白治疗的 32 名患者。适应证包括皮肌炎,包括无肌病性和副肿瘤性形式以及重叠综合征(n=18)、寻常型天疱疮(n=2)、黏膜类天疱疮(n=2)、线状 IgA 皮肤病(n=1)、坏死性血管炎(n=1)、荨麻疹性血管炎(n=1)、系统性硬皮病(n=1)、史蒂文斯-约翰逊综合征(n=1)、坏疽性脓皮病(n=3)和类脂质渐进性坏死(n=2)。该病例系列的数据证实了静脉注射免疫球蛋白作为辅助治疗选择,对于治疗抵抗的罕见免疫性皮肤病是有效且耐受良好的。