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类脂性渐进性坏死

Necrobiosis lipoidica

ORPHA:542592疾病

定义 英文原文(暂无中文)

A rare skin disease characterized by enlarging, annular plaques with red-brown edges and atrophic, yellow-brown, telangiectatic centers. The lesions are commonly asymptomatic, but affected skin areas may be fragile, and painful ulcerations develop in many cases. In rare cases, development of squamous cell carcinoma within longstanding lesions has been reported. The lower legs, especially the shins, are the most frequently involved site. The condition is often associated with diabetes mellitus.

别名

Oppenheim-Urbach疾病

基本事实

遗传方式
不适用
发病年龄
青少年期、成年期、儿童期、老年期

临床表型 21

极常见 99–80%2

  • 环状皮肤病变 HP:0025528
  • 皮肤炎症反应 HP:0011123

常见 79–30%13

  • 中性粒细胞生理功能异常 HP:0011990
  • 下肢异常 HP:0002814
  • 萎缩性瘢痕 HP:0001075
  • 糖尿病 HP:0000819
  • 红斑 HP:0010783
  • 敏感性皮肤 HP:0001030
  • 肉芽肿 HP:0032252
  • 硬化性结节 HP:0032217
  • 丘疹 HP:0200034
  • 皮肤结节 HP:0200036
  • 皮肤斑块 HP:0200035
  • 皮肤溃疡 HP:0200042
  • 皮肤毛细血管扩张 HP:0100585

偶见 29–5%4

  • 脸部异常 HP:0000271
  • 前臂形态异常 HP:0002973
  • 手异常 HP:0001155
  • 头皮形态异常 HP:0001965

罕见 <4–1%2

  • 甲状腺异常 HP:0000820
  • 鳞状细胞癌 HP:0002860

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)