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血浆置换治疗对传统治疗无反应的难治性中毒性表皮坏死松解症:一例报告及文献综述

Plasmapheresis for refractory toxic epidermal necrolysis unresponsive to conventional therapy: a case report and literature review.

作者信息

Liao Shun-Qi, Yan Zhang-Rong, Lin Lun-Wei, Deng Ming, Xiao Guo-Jin, Gao Pei-Yang

机构信息

Department of Critical Care Medicine, Hospital of Chengdu University of Traditional Chinese Medicine, Chengdu, China.

Nursing Department, Luzhou Hospital of Traditional Chinese Medicine, Luzhou, China.

出版信息

Front Immunol. 2025 May 28;16:1579349. doi: 10.3389/fimmu.2025.1579349. eCollection 2025.

Abstract

Toxic epidermal necrolysis (TEN) is a rare and life-threatening severe cutaneous adverse reaction. The conventional treatment approach includes immunomodulatory therapies, such as systemic corticosteroids, cyclosporine, intravenous immunoglobulin (IVIG), and tumor necrosis factor-alpha (TNF-α) inhibitors. Plasmapheresis, as a potential treatment for TEN, is rarely used in patients with refractory TEN. We report a successful case of plasmapheresis treatment in a patient with refractory TEN who did not respond to conventional treatment, and we provide a literature review. A 65-year-old female presented with diffuse erythematous papules covering her entire body, along with multiple blisters and bullae, and partial detachment of the epidermis and mucosa. The area of epidermal exfoliation exceeded 30% of the total body surface area, and Nikolsky's sign was positive. Despite a week of methylprednisolone treatment, numerous blisters and bullae developed, and the area of epidermal exfoliation expanded to 62%. IVIG and TNF-α inhibitors were subsequently added, but the disease remained uncontrolled. Plasmapheresis treatment was initiated. Epithelial regeneration was observed after three days of plasmapheresis. After plasmapheresis was given 5 times, the patient finally recovered. This case highlights the significance of plasmapheresis in the treatment of refractory TEN, particularly when conventional therapies are ineffective. More studies are needed in the future to confirm the efficacy of plasmapheresis treatment.

摘要

中毒性表皮坏死松解症(TEN)是一种罕见且危及生命的严重皮肤不良反应。传统治疗方法包括免疫调节疗法,如全身用皮质类固醇、环孢素、静脉注射免疫球蛋白(IVIG)和肿瘤坏死因子-α(TNF-α)抑制剂。血浆置换作为TEN的一种潜在治疗方法,在难治性TEN患者中很少使用。我们报告了1例难治性TEN患者经血浆置换治疗成功的病例,该患者对传统治疗无反应,并且我们提供了文献综述。一名65岁女性全身出现弥漫性红斑丘疹,伴有多个水疱和大疱,表皮和黏膜部分剥脱。表皮剥脱面积超过体表面积的30%,尼氏征阳性。尽管给予甲泼尼龙治疗1周,但仍出现大量水疱和大疱,表皮剥脱面积扩大至62%。随后加用IVIG和TNF-α抑制剂,但病情仍未得到控制。于是开始进行血浆置换治疗。血浆置换3天后观察到上皮再生。给予5次血浆置换后,患者最终康复。该病例突出了血浆置换在难治性TEN治疗中的重要性,尤其是在传统疗法无效时。未来需要更多研究来证实血浆置换治疗的疗效。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a117/12152979/5c4647e0c8af/fimmu-16-1579349-g001.jpg

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