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Corticosteroids plus Intravenous Immunoglobulin in the Treatment of 7 Cases with Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis
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摘要:
目的 探讨Stevens-Johnson综合征(Stevens-Johnson syndrome, SJS)和中毒性表皮松解症(toxic epidermal necrolysis, TEN)的临床特征及治疗方法, 以提高对SJS/TEN的认识。 方法 回顾性分析北京协和医院7例SJS/TEN患者的临床表现、疾病严重程度、眼部受累程度、致敏药物、治疗方案及临床结局。 结果 患者年龄26~81岁, 均伴有黏膜损害, 致敏药物以非甾体类抗炎药、抗惊厥药及别嘌醇为主, 7例均使用大剂量糖皮质激素联合人免疫球蛋白治疗, 治愈6例, 死亡1例。 结论 SJS/TEN是一组累及全身皮肤黏膜严重威胁患者生命的疾病, 病死率高, 早期足量使用大剂量糖皮质激素联合人免疫球蛋白为主要的治疗手段, 伴有系统疾病的患者预后较差。眼部损伤与疾病严重程度具有不同步性, 需及早给予针对性治疗。 -
关键词:
- Stevens-Johnson综合征 /
- 中毒性表皮松解症 /
- 糖皮质激素 /
- 人免疫球蛋白
Abstract:Objective To summarize the clinical features and treatment of Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN). Methods Seven patients with SJS/TEN were included in the study. The clinical data including severity of illness, eye involvement, causative drugs, treatment, and prognosis were retrospectively analyzed. Results The age of these patients ranged 26 to 81 years. Mucous membranes were affected in all cases. The common agents triggering SJS/TEN in these patients were nonsteroidal anti-inflammatory drugs (NSAIDs), antiepileptic drugs, and allopurinol. All these seven patients were then treated with corticosteroids plus intravenous immunoglobulin (IVIG) for their SJS/TEN. The skin lesions were healed in 6 cases, and 1 death occurred. Conclusions SJS/TEN is a life-threatening exfoliating disease of the skin and mucous membranes, and the mortality can be high without proper treatment. The use of adequate dose of corticosteroids plus IVIG is a main treatment for SJS/TEN, although the prognosis can be poor in patients with systemic diseases. Since ocular involvement in the acute stage may not relate to the severity of SJS/TEN, early ophthalmic assessment and proper treatment is warranted. -
图 1 Stevens-Johnson综合征/中毒性表皮松解症急性期患者皮肤损伤和眼部损伤
A.唇部糜烂、血痂，面颈部红斑基础上可见松弛性大疱; B.背部皮肤大片坏死松解，表皮分离呈湿烟纸样外观，Asboe-Hansen征阳性; C.生殖器黏膜糜烂; D.足背红斑中心色暗，似靶样外观; E.假性睑球黏连
表 1 Stevens-Johnson综合征/中毒性表皮松解症临床特征
临床特征 SJS SJS-TEN重叠 TEN 原发皮损 暗黑或暗红平坦非典型靶形 暗黑或暗红平坦非典型靶形 边界不清的红斑块 表皮分离:自发或摩擦后暗红皮损 平坦非典型靶形 分布 孤立皮损 孤立皮损 孤立皮损(少见) 面部和躯干融合(+) 面部和躯干融合(+ +) 面部和躯干融合(+ + +) 黏膜受累 有 有 有 系统症状 常有 均有 均有 表皮分离 ＜10%体表面积 10% ~ 30%体表面积 ＞30%体表面积 SJS: Stevens-Johnson综合征; TEN:中毒性表皮松解症 
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表 2 中毒性表皮松解症预后评分系统[5]
预后因素 SCORTEN评分 SCORTEN评分 死亡率(%) 年龄＞40岁 1 0 ~ 1 3. 2 心率＞120次/min 1 2 12. 1 癌或血液肿瘤 1 3 35. 8 首日体表受累面积超过10% 1 4 58. 3 尿素氮＞10 mmol /L 1 ≥5 90. 0 碳酸氢盐水平＜20 mmol /L 1 血糖＞14 mmol /L 1
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表 3 Stevens-Johnson综合征/中毒性表皮松解症患者致敏药物
病例 药物分类 致敏药物 1 抗惊厥药 卡马西平 2 拉莫三嗪，丙戊酸钠，丙戊酸镁 3 非甾体类抗炎药 对乙酰氨基酚、美敏伪麻溶液 4 尼美舒利 5 氨基比林，安替比林 6 具体药名不详 7 别嘌醇 别嘌醇
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