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别嘌醇诱发的中毒性表皮坏死松解症。

Allopurinol-Induced Toxic Epidermal Necrolysis.

作者信息

Perdigão Sofia, Alves Ana Sofia, Nunes Mariana, Sousa Cristiana, Barros Nelson

机构信息

Internal Medicine, Centro Hospitalar de Trás-os-Montes e Alto Douro (Hospital Center of Trás-os-Montes and Alto Douro), Chaves, PRT.

Intensive Care Unit, Centro Hospitalar de Trás-os-Montes e Alto Douro (Hospital Center of Trás-os-Montes and Alto Douro), Vila Real, PRT.

出版信息

Cureus. 2024 Jan 13;16(1):e52222. doi: 10.7759/cureus.52222. eCollection 2024 Jan.

DOI:10.7759/cureus.52222
PMID:38347997
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10861211/
Abstract

Toxic epidermal necrolysis (TEN) is a rare and life-threatening cutaneous disease, frequently triggered by drugs. Allopurinol is one of the most frequent drugs associated with TEN, which implies detachment of a significant amount of the body surface area (BSA) and has a high morbidity and mortality associated with it. We present the case of a 68-year-old female with a recent diagnosis of hyperuricemia who started treatment with allopurinol. A week later, she presented to the emergency department with an extensive maculopapular exanthema with blisters and skin detachment. After the exclusion of other etiologies, the diagnosis of allopurinol-induced TEN was made, with 35% of BSA involvement. Due to the severity of the clinical condition, she was admitted to intensive care and treated with corticoids that had no response. So, she was started on immunoglobulins and transferred to a burn unit. She developed sepsis with multiorgan failure and required supportive treatment. She was discharged after a month, and physical rehabilitation was needed. This clinical case highlights the severity of allopurinol hypersensitivity that may happen and the importance of an accurate diagnosis and treatment for this rare disease.

摘要

中毒性表皮坏死松解症(TEN)是一种罕见且危及生命的皮肤病,常由药物引发。别嘌醇是与TEN相关最常见的药物之一,这意味着身体表面积(BSA)的很大一部分会脱落,且与之相关的发病率和死亡率都很高。我们报告一例68岁女性病例,该患者近期诊断为高尿酸血症,开始使用别嘌醇治疗。一周后,她因广泛的斑丘疹、水疱和皮肤脱落就诊于急诊科。排除其他病因后,诊断为别嘌醇诱发的TEN,累及35%的BSA。由于临床病情严重,她被收入重症监护病房,接受皮质类固醇治疗但无反应。于是,她开始使用免疫球蛋白治疗并转入烧伤科。她出现败血症并伴有多器官功能衰竭,需要支持治疗。一个月后她出院,需要进行身体康复治疗。该临床病例凸显了可能发生的别嘌醇超敏反应的严重性以及对这种罕见疾病进行准确诊断和治疗的重要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d804/10861211/46b2b4452090/cureus-0016-00000052222-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d804/10861211/2fb24b433985/cureus-0016-00000052222-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d804/10861211/dfd3d3bad06c/cureus-0016-00000052222-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d804/10861211/46b2b4452090/cureus-0016-00000052222-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d804/10861211/2fb24b433985/cureus-0016-00000052222-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d804/10861211/dfd3d3bad06c/cureus-0016-00000052222-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d804/10861211/46b2b4452090/cureus-0016-00000052222-i03.jpg

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Clin Pharmacol. 2023 Oct 2;15:99-105. doi: 10.2147/CPAA.S427714. eCollection 2023.
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Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: A Review of Diagnosis and Management.史蒂文斯-约翰逊综合征和中毒性表皮坏死松解症:诊断与治疗的综述。
Medicina (Kaunas). 2021 Aug 28;57(9):895. doi: 10.3390/medicina57090895.
3
Treating toxic epidermal necrolysis with systemic immunomodulating therapies: A systematic review and network meta-analysis.
采用全身免疫调节疗法治疗中毒性表皮坏死松解症:系统评价和网络荟萃分析。
J Am Acad Dermatol. 2021 Feb;84(2):390-397. doi: 10.1016/j.jaad.2020.08.122. Epub 2020 Sep 6.
4
Erythema multiforme, Stevens-Johnson syndrome/toxic epidermal necrolysis - diagnosis and treatment.多形红斑、史蒂文斯-约翰逊综合征/中毒性表皮坏死松解症 - 诊断和治疗。
J Dtsch Dermatol Ges. 2020 Jun;18(6):547-553. doi: 10.1111/ddg.14118. Epub 2020 May 29.
5
Nikolsky's sign: A pathognomic boon.尼氏征:一种具有诊断价值的体征。
J Family Med Prim Care. 2020 Feb 28;9(2):526-530. doi: 10.4103/jfmpc.jfmpc_889_19. eCollection 2020 Feb.
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Allopurinol-Induced Toxic Epidermal Necrolysis.别嘌醇诱发的中毒性表皮坏死松解症
Drug Saf Case Rep. 2019 Sep 23;6(1):8. doi: 10.1007/s40800-019-0101-z.
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Allopurinol-induced toxic epidermal necrolysis featuring almost 60% skin detachment.别嘌醇诱发的中毒性表皮坏死松解症,皮肤剥脱面积近60%。
Medicine (Baltimore). 2019 Jun;98(25):e16078. doi: 10.1097/MD.0000000000016078.
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UK guidelines for the management of Stevens-Johnson syndrome/toxic epidermal necrolysis in adults 2016.2016年英国成人史蒂文斯-约翰逊综合征/中毒性表皮坏死松解症管理指南
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