国际眼科纵览

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Stevens-Johnson综合征和中毒性表皮松解症的病理损害、眼部表现及治疗进展

马雪燕 高华   

  1. 250000 济南, 山东省眼科医院 山东省眼科研究所(高华);250000 济南大学 山东省医学科学院医学与生命科学学院(马雪燕系在读硕士研究生); 252000 山东聊城,聊城市光明眼科医院(马雪燕)
  • 收稿日期:2015-09-09 出版日期:2016-02-22 发布日期:2016-03-07
  • 通讯作者: 高华,Email:gaohua100@126.com E-mail:gaohua100@126.com
  • 基金资助:

    国家自然科学基金(81370989,81570821);山东泰山学者计划(20081148);山东省优秀创新团队资助项目(TD201208)

Pathology, ophthalmic manifestations, and treatment of Stevens-Johnson syndrome and toxic epidermal necrolysis

MA Xue-yan 2,3, GAO Hua1   

  1.  1. Shandong Eye Hospital, Shandong Eye Institute, Jinan 250000, China; 2.  Shandong Academy of Medical Institute of Medicine and Life Science, Jinan University, Jinan 250000, China; 3. Liaocheng Guangming Eye Hospital, Shandong, Liaocheng 252000, China
  • Received:2015-09-09 Online:2016-02-22 Published:2016-03-07
  • Contact: GAO Hua, Email:gaohua100@126.com E-mail:gaohua100@126.com
  • Supported by:

    National Natural Science Foundation of China(81370989, 81570821); Shandong Taishan Scholar Program  Phase (20081148); Shandong Provincial Excellent Innovation Team Program(TD201208)

摘要:

Stevens-Johnson综合征(Stevens-Johnson syndrome,SJS)及中毒性表皮(坏死)松解症(toxic epidermal necrolysis,TEN)这两种药物不良反应能严重威胁到患者的生命安全,其均由机体免疫反应引起,两者临床症状非常相似,且互有交叉。两者均表现出全身不适、高热、皮肤疱疹及斑丘疹,常伴有黏膜损伤以及眼部损伤等。SJS与TEN引起的眼部损伤临床治疗棘手,治疗不及时容易致盲且预后差。主要病理环节为外周血液中角化细胞表面所表达的Fas受体与单个核细胞分泌的可溶性Fas配体之间发生相互作用,使角化细胞迅速凋亡、表皮损伤,临床症状逐渐明显。治疗方法包括一般性治疗(系统支持治疗、皮肤黏膜分泌物或伪膜清理、他克莫司等)和特异性治疗(如糖皮质激素、环磷酰胺等特异性药物)和眼部损伤治疗(视病情给予羊膜移植、结膜瓣覆盖、角膜移植、人工角膜及眼表重建等手术治疗)。

Abstract:

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN)  are two kinds of severe drugs adverse reactions,  which can seriously threaten the life of the patients. The clinical symptoms of the two diseases are very similar, and they are cross sometimes in clinic. The common symptoms of the two diseses are general malaise, high fever, skin herpes and spot, and often accompanied by mucosal damage and eye injury. Clinical treatment of the eye injury caused by SJS and TEN is difficult, blindness and poor prognosis are common if there is no timely treatment. The main pathogenesis is the interaction between Fas receptors and soluble Fas ligands secreted by mononuclear cells in the peripheral blood, so that the apoptosis of keratinocytes is rapidly and the  epidermal cells is damaged, and the clinical symptoms are obvious. The clinical treatment methods include general treatment (system support treatment, skin and mucosal secretions or pseudo membrane cleaning, and Tacrolimus) and specific treatment (corticosteroids, cyclophosphamide) and the treatment of eye damage (depending on the condition to give amniotic membrane transplantation and conjunctival flap covering, corneal transplantation, artificial cornea and ocular surface reconstruction surgery).