Paulmann Maren, Mockenhaupt Maja
Dokumentationszentrum Schwerer Hautreaktionen (dZh), Department of Dermatology, Medical Center - University of Freiburg, Hauptstrasse 7, 79104 Freiburg, Germany.
Curr Pharm Des. 2016;22(45):6852-6861. doi: 10.2174/1381612822666160928125152.
Severe cutaneous adverse reactions (SCAR) are known for a high morbidity and mortality. They may be life-threatening for the affected patient and difficult to accomplish for the patient's family and the treating physician. Such conditions include not only bullous reactions like toxic epidermal necrolysis (TEN) and Stevens-Johnson syndrome (SJS), but also acute generalized exanthematous pustulosis (AGEP) and drug reaction with eosinophilia and systemic symptoms (DRESS). Since clinical pattern, etiology, prognosis and treatment differ among these severe skin reactions, a clear diagnosis based on a comprehensive clinical examination, skin biopsy, and specific laboratory tests is necessary. Because most of these reactions are caused by drug intake, a thorough history of medication use has to be obtained. However, there are cases with an infectious or idiopathic cause. In any case it is crucial to identify the most likely cause and rapidly discontinue the inducing agent, if a drug cause is suspected. This is associated with the patient`s prognosis which is often poor for bullous reaction. In addition, patient's age, underlying conditions, and the extent of skin detachment play a major role in terms of prognosis. Severe cutaneous adverse reactions are T-cell-mediated reactions, and certain alleles of human leukocyte antigens (HLA) are involved in the activation of T-cells with cytotoxic effect. The therapeutic options depend on the clinical diagnosis. For all reactions a symptomatic and adequate supportive therapy is necessary, in some cases a systemic immunomodulating therapy can be useful.
严重皮肤不良反应（SCAR）以高发病率和高死亡率而闻名。它们可能对受影响的患者构成生命威胁，对患者家属和治疗医生来说也难以应对。这些情况不仅包括大疱性反应，如中毒性表皮坏死松解症（TEN）和史蒂文斯-约翰逊综合征（SJS），还包括急性泛发性脓疱病（AGEP）以及伴有嗜酸性粒细胞增多和全身症状的药物反应（DRESS）。由于这些严重皮肤反应在临床模式、病因、预后和治疗方面存在差异，因此基于全面的临床检查、皮肤活检和特定实验室检查进行明确诊断是必要的。由于这些反应大多由药物摄入引起，所以必须详细了解用药史。然而，也有一些病例是由感染或特发性原因引起的。在任何情况下，如果怀疑是药物原因，确定最可能的病因并迅速停用诱发药物至关重要。这与患者的预后相关，大疱性反应的预后通常较差。此外，患者的年龄、基础疾病以及皮肤剥脱的程度在预后方面起着重要作用。严重皮肤不良反应是T细胞介导的反应，人类白细胞抗原（HLA）的某些等位基因参与了具有细胞毒性作用的T细胞的激活。治疗方案取决于临床诊断。对于所有反应，对症和充分的支持治疗是必要的，在某些情况下，全身免疫调节治疗可能会有所帮助。
