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Abstract：
OBJECTIVE: 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.
METHODS: 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.
CONCLUSIONS: 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.
METHODS: 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.
CONCLUSIONS: 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.
摘要：
目的：大剂量静脉注射免疫球蛋白（IVIg）治疗严重的皮肤自身免疫性疾病和中毒性表皮坏死松解症（TEN）是皮肤病学中公认的方法。由于IVIg治疗通常考虑用于罕见的临床实体或严重病例，免疫球蛋白的使用通常不基于循证医学通常需要的随机对照试验的数据.由于使用IVIg的适应症很少，在可预见的将来，此类研究不太可能提供。因为一线使用受到IVIg高成本的限制，首个关于在皮肤病中使用IVIg的临床指南于2008年制定,2011年更新.
方法：这里介绍的欧洲指南是由EDF和EADV提名的专家小组编写的。制定该指南是为了更新目前认为有效的治疗适应症，并总结在皮肤自身免疫性疾病和TEN中使用IVIg的证据。
结论：当前的指南代表了关于IVIg使用的专家意见和定义，反映了当前已发表的证据，旨在作为在皮肤病中使用IVIg的决策工具。
方法：这里介绍的欧洲指南是由EDF和EADV提名的专家小组编写的。制定该指南是为了更新目前认为有效的治疗适应症，并总结在皮肤自身免疫性疾病和TEN中使用IVIg的证据。
结论：当前的指南代表了关于IVIg使用的专家意见和定义，反映了当前已发表的证据，旨在作为在皮肤病中使用IVIg的决策工具。
