增殖型天疱疮
Pemphigus vegetans
ORPHA:79479疾病
定义 英文原文（暂无中文）
A rare autoimmune bullous skin disease characterized by mucocutaneous bullae with subsequent erosion and formation of vegetative plaques, predominantly affecting intertriginous areas and the oral mucosa. Two clinical forms of the disease are recognized: the Hallopeau type, which presents an indolent course with pustules healing as vegetative plaques and frequent lack of involvement of the oral mucosa, and the Neumann type, which takes a more severe, refractory course with vegetations developing during an eruption of vesiculobullous lesions and involvement of the oral mucosa. Serum analysis reveals antibodies against desmoglein 1 and 3.
基本事实
- 发病年龄
- 各年龄段
- 患病率
- 1-9 / 1 000 000（Germany）
临床表型 25
极常见 99–80%2
- 抗桥粒黏蛋白-1抗体阳性 HP:4000013
- 抗桥粒黏蛋白-3抗体阳性 HP:4000014
常见 79–30%17
- 唇形态异常 HP:0000159
- 舌头形态异常 HP:0030809
- 抗桥粒芯蛋白抗体阳性 HP:6000448
- 肉芽组织旺盛 HP:6000956
- 角化过度 HP:0000962
- 表皮内起疱 HP:0033802
- 皮肤浸渍 HP:0032007
- 口腔粘膜水泡 HP:0200097
- 疼痛 HP:0012531
- 丘疹疱疹 HP:0033700
- 脓疱 HP:0200039
- 皮肤糜烂 HP:0200041
- 皮肤斑块 HP:0200035
- 皮肤溃疡 HP:0200042
- 口腔炎 HP:0010280
- 疣状丘疹 HP:0012500
- 水疱疹 HP:0033697
偶见 29–5%6
- 指（趾）甲形态异常 HP:0001597
- 甲面异常 HP:0025757
- 脆甲 HP:0001808
- 营养不良 HP:0004395
- 脱甲病 HP:0025088
- 甲沟炎 HP:0001818
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）