Sweet综合征
Sweet syndrome
ORPHA:3243疾病
定义 英文原文（暂无中文）
A rare inflammatory disease characterized by abrupt appearance of painful, edematous and erythematous papules, plaques and nodules on the skin, and frequently accompanied by fever and neutrophilia with a dense infiltration of mature neutrophils that are typically located in the upper dermis. The disease is classically associated with inflammatory disease, pregnancy, infection (mostly of the upper respiratory tract), or vaccination but may be idiopathic, associated with a hematological or visceral malignancy, or drug-induced.
别名
急性发热性嗜中性皮肤病
基本事实
- 遗传方式
- 多基因/多因素
- 发病年龄
- 成年期
相关基因 2
| 基因 | 名称 | 关联类型 |
|---|---|---|
| MEFV | MEFV innate immunity regulator, pyrin | Disease-causing germline mutation(s) in |
| PTPN6 | protein tyrosine phosphatase non-receptor type 6 | Major susceptibility factor in |
临床表型 39
极常见 99–80%6
- 循环白细胞介素水平异常 HP:0011117
- 红色丘疹 HP:0030350
- 红斑 HP:0025474
- 疼痛 HP:0012531
- 嗜中性粒细胞为主真皮浸润 HP:0031236
- 皮肤结节 HP:0200036
常见 79–30%16
- 脸部异常 HP:0000271
- 手异常 HP:0001155
- 颈部异常 HP:0000464
- 肿瘤坏死因子分泌异常 HP:0011118
- 痤疮 HP:0001061
- 关节疼痛 HP:0002829
- C-反应蛋白水平升高 HP:0011227
- 红细胞沉降速度升高 HP:0003565
- 循环白细胞介素6水平升高 HP:0030783
- 白细胞增多症 HP:0001974
- 肌痛 HP:0003326
- 肌炎 HP:0100614
- 中性粒细胞增多症 HP:0011897
- 非周期性反复发热 HP:0032324
- 小血管炎 HP:0011944
- 无菌性脓肿 HP:0025616
偶见 29–5%14
- 皮肤的异常起疱 HP:0008066
- 药物反应异常 HP:0020169
- 化脓性汗腺炎 HP:0040154
- 急性髓性白血病 HP:0004808
- 贫血 HP:0001903
- 血液肿瘤 HP:0004377
- 大肠炎症 HP:0002037
- 肿瘤 HP:0002664
- 寡关节炎 HP:0040313
- 脂膜炎 HP:0012490
- 脓疱 HP:0200039
- 坏疽性脓皮病 HP:0025452
- 反复感染 HP:0002719
- 表皮水疱 HP:0200037
罕见 <4–1%3
- 乳腺癌 HP:0003002
- 慢性淋巴细胞白血病 HP:0005550
- 扩张型心肌病 HP:0001644
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）