Enk Alexander, Hadaschik Eva, Eming Rüdiger, Fierlbeck Gerhard, French Lars, Girolomoni Giampiero, Hertl Michael, Jolles Stephen, Karpati Sarolta, Steinbrink Kerstin, Stingl Georg, Volc-Platzer Beatrix, Zillikens Detlef
Department of Dermatology, Ruprecht-Karls-University Heidelberg, Germany.
Department of Dermatology, Philipps-University Marburg, Germany.
J Dtsch Dermatol Ges. 2017 Feb;15(2):228-241. doi: 10.1111/ddg.13013. Epub 2016 Dec 30.
Treatment of severe dermatological autoimmune diseases and toxic epidermal necrolysis (TEN) with high-dose intravenous immunoglobulin (IVIg) is a well-established procedure in dermatology. As treatment with IVIg is usually considered for rare clinical entities or severe cases, the use of immunoglobulin is not generally based on data from randomized controlled trials usually required for evidence-based medicine. Since the indications for the use of IVIg are rare, it is unlikely that such studies will be available in the foreseeable future. Because first-line use is limited by the high costs of IVIg, the first clinical guidelines on the use of IVIg in dermatological conditions were established in 2008 and renewed in 2011.
The European guidelines presented here were prepared by a panel of experts nominated by the EDF and EADV. The guidelines were developed to update the indications for treatment currently considered effective and to summarize the evidence for the use of IVIg in dermatological autoimmune diseases and TEN.
The current guidelines represent consensual expert opinions and definitions on the use of IVIg reflecting current published evidence and are intended to serve as a decision-making tool for the use of IVIg in dermatological diseases.
大剂量静脉注射免疫球蛋白（IVIg）治疗严重皮肤自身免疫性疾病和中毒性表皮坏死松解症（TEN）是皮肤科既定的治疗方法。由于通常针对罕见临床病症或重症病例使用IVIg治疗，免疫球蛋白的使用一般并非基于循证医学通常所需的随机对照试验数据。鉴于IVIg的使用指征罕见，在可预见的未来不太可能有此类研究。由于IVIg的高昂成本限制了其一线使用，2008年制定了关于IVIg在皮肤病中使用的首批临床指南，并于2011年进行了更新。
此处呈现的欧洲指南由法国皮肤病学会（EDF）和欧洲皮肤病与性病学会（EADV）提名的专家小组制定。制定这些指南是为了更新目前被认为有效的治疗指征，并总结IVIg在皮肤自身免疫性疾病和TEN中使用的证据。
当前指南代表了关于IVIg使用的共识性专家意见和定义，反映了当前已发表的证据，旨在作为IVIg在皮肤病中使用的决策工具。
