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中毒性表皮坏死松解症:一种危重症范例。

Toxic epidermal necrolysis: a paradigm of critical illness.

作者信息

Estrella-Alonso Alfonso, Aramburu José Antonio, González-Ruiz Mercedes Yolanda, Cachafeiro Lucía, Sánchez Manuel Sánchez, Lorente José A

机构信息

Instituto de Investigación Sanitaria del Hospital Universitario de Getafe - Madrid, Spain.

Universidad Europea - Madrid, Spain.

出版信息

Rev Bras Ter Intensiva. 2017 Oct-Dec;29(4):499-508. doi: 10.5935/0103-507X.20170075.

Abstract

Toxic epidermal necrolysis is an adverse immunological skin reaction secondary in most cases to the administration of a drug. Toxic epidermal necrolysis, Stevens-Johnson syndrome, and multiform exudative erythema are part of the same disease spectrum. The mortality rate from toxic epidermal necrolysis is approximately 30%. The pathophysiology of toxic epidermal necrolysis is similar in many respects to that of superficial skin burns. Mucosal involvement of the ocular and genital epithelium is associated with serious sequelae if the condition is not treated early. It is generally accepted that patients with toxic epidermal necrolysis are better treated in burn units, which are experienced in the management of patients with extensive skin loss. Treatment includes support, elimination, and coverage with biosynthetic derivatives of the skin in affected areas, treatment of mucosal involvement, and specific immunosuppressive treatment. Of the treatments tested, only immunoglobulin G and cyclosporin A are currently used in most centers, even though there is no solid evidence to recommend any specific treatment. The particular aspects of the treatment of this disease include the prevention of sequelae related to the formation of synechiae, eye care to prevent serious sequelae that can lead to blindness, and specific immunosuppressive treatment. Better knowledge of the management principles of toxic epidermal necrolysis will lead to better disease management, higher survival rates, and lower prevalence of sequelae.

摘要

中毒性表皮坏死松解症是一种不良免疫性皮肤反应,多数情况下继发于药物使用。中毒性表皮坏死松解症、史蒂文斯-约翰逊综合征和多形性渗出性红斑属于同一疾病谱。中毒性表皮坏死松解症的死亡率约为30%。中毒性表皮坏死松解症的病理生理学在许多方面与浅表皮肤烧伤相似。如果病情未得到早期治疗,眼部和生殖器上皮的黏膜受累会伴有严重的后遗症。普遍认为,中毒性表皮坏死松解症患者在烧伤病房接受治疗效果更佳,烧伤病房在处理大面积皮肤缺损患者方面经验丰富。治疗包括支持治疗、清除坏死组织、用皮肤生物合成衍生物覆盖受影响区域、治疗黏膜受累以及进行特异性免疫抑制治疗。在已测试的治疗方法中,目前大多数中心仅使用免疫球蛋白G和环孢素A,尽管尚无确凿证据推荐任何特定治疗方法。该疾病治疗的特殊方面包括预防与粘连形成相关的后遗症、眼部护理以预防可能导致失明的严重后遗症以及特异性免疫抑制治疗。对中毒性表皮坏死松解症管理原则的更好了解将带来更好的疾病管理、更高的生存率以及更低的后遗症发生率。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/fbba/5764563/612e7f698a34/rbti-29-04-0499-g01.jpg

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