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一名患有狼疮相关性胰腺炎的儿童发生中毒性表皮坏死松解症。

Toxic epidermal necrolysis in a child with lupus-associated pancreatitis.

作者信息

Marija Stevic, Ivana Budic, Nina Ristic, Dragan Nenadic, Zlatko Bokun, Branislav Jovanovic, Jelena Pejanovic, Dusica Simic

机构信息

Department of Anesthesia, Faculty of Medicine, University of Belgrade, Tirsova 10, Belgrade, Serbia.

Department of Anesthesia, Faculty of Medicine, Clinical Centre Nis, University of Nis, Bulevar dr Zorana Djindjica 81, Nis, Serbia.

出版信息

Rheumatol Int. 2017 Jul;37(7):1221-1226. doi: 10.1007/s00296-017-3677-6. Epub 2017 Feb 26.

Abstract

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threatening dermatological conditions that are characterized by mucosal erosions, epidermal detachments and erosions. The most common causes of SJS and TEN are drugs; other causes such as systemic lupus erythematosus (SLE), vaccinations and infections have been rarely implicated. We present the case of a 14-year-old female patient with acute pancreatitis as an initial manifestation of systemic lupus erythematosus, complicated by the toxic epidermal necrolysis with a fatal outcome. She initially presented with abdominal pain, fever, vomiting, and intolerance to oral intake and elevated pancreatic enzyme levels. Systemic lupus erythematosus was diagnosed secondary when her condition has been already complicated by the toxic epidermal necrolysis. The administration of corticosteroids and high doses of intravenous immunoglobulin did not lead to positive effects in the treatment of our patient.

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)是危及生命的皮肤病,其特征为黏膜糜烂、表皮剥脱和溃疡。SJS和TEN最常见的病因是药物;其他病因如系统性红斑狼疮(SLE)、疫苗接种和感染很少涉及。我们报告一例14岁女性患者,以急性胰腺炎为系统性红斑狼疮的初始表现,并发中毒性表皮坏死松解症,最终死亡。她最初表现为腹痛、发热、呕吐、不耐受口服摄入以及胰酶水平升高。当她的病情已因中毒性表皮坏死松解症而复杂化时,才继发性诊断出系统性红斑狼疮。给予皮质类固醇和大剂量静脉注射免疫球蛋白对我们这位患者的治疗未产生积极效果。

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