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离心性脂肪代谢障碍

Centrifugal lipodystrophy

ORPHA:90156疾病

定义 英文原文(暂无中文)

Centrifugal lipodystrophy is a rare, acquired, localized lipodistrophy characterized by single or, occasionally, multiple, centrifugally progressive, asymptomatic to sometimes mildly tender, hypopigmented, lipoatrophic skin depressions with weakly erymatheous inflammatory borders, typically associated with regional ipsilateral lymph nodes swelling. Lesions typically occur on lower trunk (in particular groin and abdomen region), followed by upper trunk (axilla and neighboring regions) and, rarely, neck and head. It is usually not associated with systemic disease and is typically self-resolving.

别名

幼儿腹部离心性脂肪营养不良

基本事实

发病年龄
青少年期、成年期、儿童期、老年期、婴儿期
患病率
<1 / 1 000 000

临床表型 10

必现 100%1

  • 脂肪萎缩 HP:0100578

极常见 99–80%1

  • 皮下脂肪组织减少 HP:0003758

常见 79–30%1

  • 皮下脂肪缺如 HP:0007485

偶见 29–5%5

  • 胸廓形态异常 HP:0000765
  • 红斑 HP:0010783
  • 皮肤炎症反应 HP:0011123
  • 淋巴结炎 HP:0002840
  • 皮肤脱屑 HP:0040189

罕见 <4–1%2

  • 脱发 HP:0001596
  • 面部皮下脂肪缺乏 HP:0005320

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)