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区别大疱性表皮松解型多形红斑与史蒂文斯-约翰逊综合征(SJS)/中毒性表皮坏死松解症(TEN)的关键因素。

Critical factors differentiating erythema multiforme majus from Stevens-Johnson syndrome (SJS)/toxic epidermal necrolysis (TEN).

机构信息

Department of Dermatology, Showa University School of Medicine, 1-5-8 Hatanodai Shinagawa-ku, Tokyo 142-8666, Japan.

出版信息

Eur J Dermatol. 2011 Nov-Dec;21(6):889-94. doi: 10.1684/ejd.2011.1510.

Abstract

Erythema multiforme majus (EMM) and Stevens-Johnson syndrome (SJS)/toxic epidermal necrolysis (TEN) are severe cutaneous reactions characterised by targetoid erythematous lesions and mucocutaneous involvement. The initial skin manifestations are similar, making early diagnosis difficult. We retrospectively reviewed 36 cases of EMM and 18 cases of SJS/TEN and also evaluated 6 patients with unclassified EMM. 13 patients in the EMM group and 16 patients in the SJS/TEN group presented with a high fever (>38.5̊C; p<0.001). Two or more mucous membranes were affected in 6 patients in the EMM group and 18 patients in the SJS/TEN group. Significantly more SJS/TEN than EMM patients had high levels of C-reactive protein and severe hepatic dysfunction. Thirteen EMM and 13 SJS/TEN cases were caused by medications/drugs. Skin biopsy samples showed stronger mononuclear cell infiltration in the EMM than in the SJS/TEN group (p<0.001). The mean dose of initial systemic corticosteroid used to treat EMM was lower than that used to treat SJS/TEN. No patients died in either group. Clinically, the unclassified cases mostly behaved like EMM. The results of our investigation suggest that EMM and SJS/TEN are distinct conditions and they help in differentiating these syndromes at an early stage.

摘要

红斑多形性大疱症(EMM)和史蒂文斯-约翰逊综合征(SJS)/中毒性表皮坏死松解症(TEN)是严重的皮肤反应,其特征为靶形红斑性病变和粘膜皮肤受累。初始皮肤表现相似,导致早期诊断困难。我们回顾性分析了 36 例 EMM 和 18 例 SJS/TEN 病例,并对 6 例未分类的 EMM 患者进行了评估。EMM 组中有 13 例和 SJS/TEN 组中有 16 例患者出现高热(>38.5°C;p<0.001)。在 EMM 组中有 6 例和 SJS/TEN 组中有 18 例患者累及两个或多个粘膜。SJS/TEN 患者的 C 反应蛋白水平和严重肝肾功能障碍显著高于 EMM 患者。13 例 EMM 和 13 例 SJS/TEN 由药物/药物引起。皮肤活检样本显示 EMM 组比 SJS/TEN 组有更强的单核细胞浸润(p<0.001)。用于治疗 EMM 的初始系统性皮质类固醇的平均剂量低于用于治疗 SJS/TEN 的剂量。两组均无患者死亡。临床上,未分类的病例大多表现为 EMM。我们的研究结果表明,EMM 和 SJS/TEN 是不同的疾病,有助于早期区分这些综合征。

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