Department of Dermatology and Venereology, University Medical Center, Rostock, Germany.
Center for the Documentation of Severe Skin Reactions (dZh), Department of Dermatology and Venereology, University Medical Center, Freiburg, Germany.
J Dtsch Dermatol Ges. 2020 Jun;18(6):547-553. doi: 10.1111/ddg.14118. Epub 2020 May 29.
Prior to the first international consensus classification published in 1993, the clinical distinction between erythema multiforme (EM), Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) had been subject to uncertainty and controversy for more than a century. Based on this classification, the three conditions are defined by the morphology of the individual lesions and their pattern of distribution. Etiopathogenetically, the majority of EM cases is caused by infections (primarily herpes simplex virus and Mycoplasma pneumoniae), whereas SJS/TEN are predominantly triggered by drugs. The SCORTEN (score of toxic epidermal necrolysis) can and should be used to assess disease prognosis in patients with SJS/TEN. While supportive treatment is generally considered sufficient for EM, there is still uncertainty as to the type of systemic therapy required for SJS/TEN. Given the lack of high-quality therapeutic trials and (in some cases) conflicting results, it is currently impossible to issue definitive recommendations for any given immunomodulatory therapy. While there is always a trade-off between rapid onset of treatment-induced immunosuppression and an uptick in infection risk, there has been increasing evidence that cyclosporine in particular may be able to halt disease progression (i.e. skin detachment) and lower mortality rates. Assistance in diagnosis and management of the aforementioned conditions may be obtained from the Center for the Documentation of Severe Skin Reactions (dZh) at the Department of Dermatology, University Medical Center, Freiburg, Germany.
在 1993 年发布的首个国际共识分类之前，多形性红斑（EM）、史蒂文斯-约翰逊综合征（SJS）和中毒性表皮坏死松解症（TEN）之间的临床区别已经存在了一个多世纪，存在不确定性和争议。基于这一分类，这三种情况是根据个体损伤的形态及其分布模式来定义的。从发病机制上讲，大多数 EM 病例是由感染（主要是单纯疱疹病毒和肺炎支原体）引起的，而 SJS/TEN 主要由药物引发。SCORTEN（中毒性表皮坏死松解症评分）可用于评估 SJS/TEN 患者的疾病预后，也应该用于评估 SJS/TEN 患者的疾病预后。虽然支持性治疗通常被认为足以治疗 EM，但对于 SJS/TEN 需要哪种类型的系统治疗仍存在不确定性。由于缺乏高质量的治疗试验和（在某些情况下）相互矛盾的结果，目前无法对任何特定的免疫调节治疗给出明确的建议。虽然治疗引起的免疫抑制作用的快速起效与感染风险的增加之间总是存在权衡，但越来越多的证据表明，环孢素尤其可能能够阻止疾病进展（即皮肤脱落）并降低死亡率。德国弗莱堡大学医学中心皮肤科的严重皮肤反应文件中心（dZh）可提供上述疾病的诊断和治疗协助。
