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重症中毒性表皮坏死松解症的口咽喉表现:一项单中心回顾性病例系列研究

Oropharyngolaryngeal manifestations in severe toxic epidermal necrolysis: a single-center's retrospective case series.

作者信息

Glasson Nicolas, De Sandre Cécile, Pantet Olivier, Reinhard Antoine, Lambercy Karma, Sandu Kishore, Gorostidi François

机构信息

Department of Otolaryngology-Head and Neck Surgery, CHUV, Lausanne, Switzerland.

Department of Adult Intensive Care, CHUV, Lausanne, Switzerland.

出版信息

Int J Dermatol. 2023 Nov;62(11):1384-1390. doi: 10.1111/ijd.16858. Epub 2023 Sep 28.

DOI:10.1111/ijd.16858
PMID:37767642
Abstract

BACKGROUND

Toxic epidermal necrolysis is a rare and life-threatening mucocutaneous disease. Although mucosal ear, nose, and throat (ENT) involvement is common, little is known about the characteristics, treatment modalities, and outcomes of these lesions. The aim of this study was to evaluate ENT mucosal lesions in severe toxic epidermal necrolysis patients and analyze their characteristics, treatment modalities, and outcomes, as well as proposing a management algorithm to prevent long-term debilitating sequalae of these lesions.

METHODS

This is a retrospective review of toxic epidermal necrolysis cases treated at the tertiary burns unit of the Lausanne University Hospital CHUV, Switzerland, between 2006 and 2019.

RESULTS

Out of 19 patients with severe toxic epidermal necrolysis, 17 (89%) underwent a complete ENT examination at admission and 14 (82%) had ENT mucosal involvement. Five (26.3%) patients died during the stay in the intensive care unit. Of the 16 patients who received maximal care, 13 (81%) required orotracheal intubation for a median time of 16 (IQR: 14) days. Out of the 14 patients who survived, four (29%) had long-term ENT complications consisting of synechiaes necessitating subsequent endoscopic procedures. Those four patients all required mechanical ventilation with an orotracheal tube and suffered from hypopharyngeal synechiaes as well as oral and endonasal synechiaes in individual cases.

CONCLUSION

This study suggests a high prevalence of ENT synechiaes in patients with severe toxic epidermal necrolysis and requiring orotracheal intubation. Periodic ENT examination could prevent mature synechiae formation in these patients. We propose an algorithm to prevent long-term sequalae in ENT mucosal involvement.

摘要

背景

中毒性表皮坏死松解症是一种罕见且危及生命的皮肤黏膜疾病。虽然耳鼻咽喉(ENT)黏膜受累很常见,但对于这些病变的特征、治疗方式及预后了解甚少。本研究的目的是评估重症中毒性表皮坏死松解症患者的ENT黏膜病变,分析其特征、治疗方式及预后,并提出一种管理算法以预防这些病变的长期致残后遗症。

方法

这是一项对2006年至2019年间在瑞士洛桑大学医院CHUV三级烧伤科治疗的中毒性表皮坏死松解症病例的回顾性研究。

结果

19例重症中毒性表皮坏死松解症患者中,17例(89%)在入院时接受了全面的ENT检查,14例(82%)有ENT黏膜受累。5例(26.3%)患者在重症监护病房住院期间死亡。在接受最大程度治疗的16例患者中,13例(81%)需要经口气管插管,中位时间为16(四分位间距:14)天。在14例存活患者中,4例(29%)有长期ENT并发症,包括粘连,需要随后进行内镜手术。这4例患者均需要经口气管插管进行机械通气,个别病例还患有下咽粘连以及口腔和鼻内粘连。

结论

本研究表明,重症中毒性表皮坏死松解症且需要经口气管插管的患者中ENT粘连的发生率很高。定期进行ENT检查可预防这些患者形成成熟粘连。我们提出一种算法以预防ENT黏膜受累的长期后遗症。

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