中毒性表皮坏死松解型药疹

Toxic Epidermal Necrolysis

中毒性表皮坏死松解型药疹是一种严重、致命的皮肤黏膜疾病,大部分由药物引起,少部分与感染相关。患者具有一定的遗传易感性,其体内的细胞毒性T细胞、NK细胞异常活化增殖后直接或间接通过分泌毒性蛋白杀死角质形成细胞。临床上主要表现为广泛的表皮剥脱、坏死,黏膜糜烂,累及面积>30%。组织学特点为表皮不同程度或全层坏死、水疱形成。治疗方面,应立即停用致敏药物,迅速进行强有力的支持治疗,系统应用糖皮质激素或静脉注射用丙种球蛋白(IVIG)、环孢素、抗肿瘤坏死因子-α(TNF-α)单克隆抗体等。

Toxic epidermal necrolysis（ TEN） is a fatal mucocutaneous disease,most of which are caused by drugs and some are associated with infection. Cytotoxic T lymphocytes and NK cells kill keratinocytes directly orindirectly by secreting toxic cytokines after activated by culprit drug in patients with genetic predisposition. Extensive epidermis exfoliation,necrosis and mucosal erosion are the characteristics of TEN that involves detachment of 〉30 percent of the body surface area. Histopathology is characterized by different degrees or full thickness epidermal necrosis. Prompt with drawal of the offending agent and support therapy are important after diagnosis. Treatment of systemic corticosteroids,Intravenous immune globulin（ IVIG）,Cyclosporineortumor necrosis factor inhibitors is applied.