Stevens-Johnson综合征
Stevens-Johnson syndrome
定义 英文原文（暂无中文）
A limited form of Stevens-Johnson syndrome/toxic epidermal necrolysis spectrum characterized by destruction and detachment of the skin epithelium, involving less than 10% of the body surface area, and mucous membranes. Onset usually occurs 4-28 days after administration of the causal medication and is most frequently associated with anticonvulsants, antibacterial sulfonamides, allopurinol, nevirapine, and oxicams (non-steroidal anti-inflammatory drugs), but many other medications have also been implicated. The disease is not induced by medication in 15% of cases. Histology is characterized by an epidermal necrolysis. Multiple disabling long-term sequelae (especially cutaneous, ocular and psychological) are frequent.
别名
多形性红斑Stevens Johnson型
基本事实
- 遗传方式
- 不适用
- 发病年龄
- 各年龄段
- 患病率
- 1-9 / 1 000 000（Europe）
相关基因 2
| 基因 | 名称 | 关联类型 |
|---|---|---|
| HLA-B | major histocompatibility complex, class I, B | Major susceptibility factor in |
| IKZF1 | IKAROS family zinc finger 1 | Major susceptibility factor in |
临床表型 39
极常见 99–80%9
- 皮肤的异常起疱 HP:0008066
- 皮肤棘层松解 HP:0100792
- 腹泻 HP:0002014
- 红斑 HP:0010783
- 疲乏 HP:0012378
- 发热 HP:0001945
- 斑疹 HP:0012733
- 恶心和呕吐 HP:0002017
- 体重减轻 HP:0001824
常见 79–30%3
- 中性粒细胞异常 HP:0001874
- 吞咽困难 HP:0002015
- 唾液分泌过多 HP:0003781
偶见 29–5%27
- 腹痛 HP:0002027
- 心肌形态异常 HP:0001637
- 胸膜异常 HP:0002103
- 尿道异常 HP:0000795
- 急性肝功能衰竭 HP:0006554
- 贫血 HP:0001903
- 结膜炎 HP:0000509
- 角膜糜烂 HP:0200020
- 咳嗽 HP:0012735
- 性交疼痛 HP:0030016
- 呼吸困难 HP:0002094
- 排尿困难 HP:0100518
- 循环肝转氨酶水平升高 HP:0002910
- 眼睑内翻 HP:0000621
- 食管狭窄 HP:0002043
- 胃肠道出血 HP:0002239
- 低钾性代谢性碱中毒 HP:0001960
- 心肌梗死 HP:0001658
- 胰腺炎 HP:0001733
- 畏光 HP:0000613
- 反复呼吸道感染 HP:0002205
- 肾功能不全 HP:0000083
- 限制性通气功能障碍 HP:0002091
- 脓毒症 HP:0100806
- 心脏性猝死 HP:0001645
- 血小板减少症 HP:0001873
- 视觉障碍 HP:0000505
外部标识与链接
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本页数据来源
- 疾病定义、同义词、基因、表型、流行病学：Orphanet（CC BY 4.0）
- 中文病名：Orphanet 中文包，冻结于 2020-06-01
- 表型中文标签：HPO 简体中文翻译（CHPO 上游成果）