红斑型天疱疮
Pemphigus erythematosus
ORPHA:79480疾病
定义 英文原文（暂无中文）
A rare superficial pemphigus disease characterized clinically by well-demarcated, localized, erythematous, scaly, hyperkeratotic, crusted plaques, with frequent butterfly distribution over the malar area of the face (but also commonly involving trunk and scalp, and less frequently the extremities, with a photoexposed distribution). Histologically, granular deposits along the dermal-epidermal junction, in addition to intercellular deposition in the upper epidermis, are observed.
别名
Senear-Usher综合征
基本事实
- 发病年龄
- 青少年期、成年期、儿童期
临床表型 14
极常见 99–80%5
- 皮肤棘层松解 HP:0100792
- 自身免疫 HP:0002960
- 红斑 HP:0025474
- 局限性皮损 HP:0011355
- 颧部皮疹 HP:0025300
常见 79–30%3
- 皮肤的异常起疱 HP:0008066
- 抗核抗体阳性 HP:0003493
- 表皮基底膜带的线性lgG沉积 HP:0031540
偶见 29–5%6
- 抗神经肌肉接头乙酰胆碱受体抗体阳性 HP:0030208
- 真皮局灶性发育缺陷/发育不全 HP:0007510
- 皮肤色素减退斑 HP:0001053
- 口腔溃疡 HP:0000155
- 皮肤糜烂 HP:0200041
- 系统性红斑狼疮 HP:0002725
外部标识与链接
发现这一页有错误？告诉我 · 邮件主题会自动带上本页的 ORPHA 编号
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）