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首页> 外文期刊>Journal of clinical rheumatology >Systemic lupus erythematosus presenting as stevens-johnson syndrome/toxic epidermal necrolysis
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Systemic lupus erythematosus presenting as stevens-johnson syndrome/toxic epidermal necrolysis

机译：系统性红斑狼疮表现为史蒂文斯-约翰逊综合征/毒性表皮坏死

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摘要

Stevens-Johnson syndrome (SJS)/toxic epidermal necrolysis (TEN)-like lesions in acute cutaneous lupus erythematosus [LE]) are an unusual manifestation of systemic LE. We describe a patient with widespread vesiculobullous lesions diagnosed as SJS/TEN-like acute cutaneous LE as the initial presentation of systemic LE. Stevens-Johnson syndrome/TEN-like LE may be differentiated from other vesiculobullous lesions by factors including a history of recent LE exacerbation, photodistribution of lesions, lack of a precipitating infection or medication exposure, minimal mucosal involvement, a prolonged course, response steroid treatment, and histologic and immunofluorescence findings. It is paramount to identify SJS/TEN-like LE as this condition requires early and aggressive intervention. The optimal treatment approach for SJS/TEN-like LE is unclear, and although some case reports have shown glucocorticoids to be useful, there are also reports of cases in which additional measures, such as intravenous immunoglobulin and plasmapheresis, were required to achieve a response. Our patient's condition was refractory to high-dose corticosteroids and intravenous immunoglobulin but was successfully treated using plasma exchange. As such, this treatment may hold potential for improving the care of other patients with refractory SJS/TEN-like LE.
机译：急性皮肤性红斑狼疮[LE]中的史蒂文斯-约翰逊综合症（SJS）/中毒性表皮坏死溶解（TEN）样病变是系统性LE的异常表现。我们将诊断为SJS / TEN样急性皮肤LE的广泛囊泡性病变的患者描述为系统性LE的最初表现。史蒂文斯-约翰逊综合症/ TEN样LE可通过以下因素与其他囊泡性病变区分开来，这些因素包括近期LE加重病史，病变的光分布，缺乏感染或药物暴露，粘膜受累最小，病程延长，类固醇治疗反应，以及组织学和免疫荧光检查结果。识别SJS / TEN样LE至关重要，因为这种情况需要尽早而积极地干预。尚不清楚SJS / TEN样LE的最佳治疗方法，尽管一些病例报告显示糖皮质激素是有用的，但也有报告称需要采取其他措施（例如静脉内免疫球蛋白和血浆置换）来达到缓解效果。我们患者的病情对大剂量皮质类固醇和静脉内免疫球蛋白均不敏感，但已通过血浆置换成功治疗。因此，这种治疗方法可能具有改善其他难治性SJS / TEN样LE病人的护理潜力。

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