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苯妥英钠诱发史蒂文斯-约翰逊综合征和闭塞性细支气管炎——病例报告及文献复习

Phenytoin induced Steven-Johnson syndrome and bronchiolitis obliterans - case report and review of literature.

作者信息

Pannu Bibek S, Egan Ashley M, Iyer Vivek N

机构信息

Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, MN, USA.

Division of General Internal Medicine, Mayo Clinic, Rochester, MN, USA.

出版信息

Respir Med Case Rep. 2016 Jan 20;17:54-6. doi: 10.1016/j.rmcr.2016.01.006. eCollection 2016.

Abstract

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are both rare but serious idiosyncratic drug reactions characterized by diffuse muco-epidermoid injury and high mortality. Keratinocytes in both skin and mucous membranes (including eyes, mouth and genitalia) are injured resulting in a diffuse maculopapular rash, blistering lesions and epithelial detachment with minimal force (Nikolsky's sign). SJS is typically diagnosed when less than 10% of the skin surface is involved and the term TEN is used in cases with more than 30% involvement. Respiratory involvement in SJS-TEN is common with 30-50% of cases demonstrating respiratory epithelial sloughing with severe short and long term complications. Patients who survive SJS-TEN are often left with impaired respiratory function and bronchiolitis obliterans. Cases of bronchiolitis obliterans with SJS/TEN have been very rarely reported. We report a case of phenytoin induced SJS/TEN followed by severe bronchiolitis obliterans in an adult patient. The presentation, pathophysiology and management of SJS/TEN related bronchiolitis obliterans is also reviewed.

摘要

史蒂文斯-约翰逊综合征(SJS)和中毒性表皮坏死松解症(TEN)均为罕见但严重的特异质性药物反应,其特征为弥漫性黏膜表皮损伤且死亡率高。皮肤和黏膜(包括眼睛、口腔和生殖器)中的角质形成细胞受损,导致弥漫性斑丘疹、水疱性损害以及轻微外力即可引起上皮剥脱(尼氏征)。当皮肤受累面积小于10%时通常诊断为SJS,而受累面积超过30%的病例则诊断为TEN。SJS-TEN常累及呼吸系统,30%-50%的病例出现呼吸道上皮脱落,并伴有严重的短期和长期并发症。SJS-TEN存活患者常遗留呼吸功能受损和闭塞性细支气管炎。SJS/TEN合并闭塞性细支气管炎的病例报道极少。我们报告1例成年患者因苯妥英钠诱发SJS/TEN,随后发生严重闭塞性细支气管炎的病例。本文还对SJS/TEN相关闭塞性细支气管炎的临床表现、病理生理学及治疗进行了综述。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/36bf/4821333/c89503477a10/gr1.jpg

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