EC20.01
X连锁鱼鳞病
X-linked ichthyosis
- 上级条目
- EC20.0 非综合征性鱼鳞病
- 层级
- 5 级
定义
X连锁鱼鳞病是一种X连锁隐性遗传性皮肤病,与类固醇硫酸酯酶缺乏和血浆硫酸胆固醇升高有关。出生时或出生后不久会出现全身性鳞屑,在四肢、颈部、躯干和臀部最为突出。它只发生在男性,可能与睾丸疾病和角膜混浊有关。
X-linked ichthyosis is an X-linked recessive genodermatosis associated with steroid sulfatase deficiency and elevated plasma cholesterol sulfate. Generalised scaling is present at or shortly after birth, most prominently over the extremities, neck, trunk, and buttocks. It occurs only in males and may be associated with testicular disease and corneal opacities.
索引词 可用于检索该条目的同义词与别名
- X连锁鱼鳞病
- 性连锁鱼鳞病
- X连锁鱼鳞病伴类固醇硫酸脂酶缺乏
- X连锁隐性遗传鱼鳞病
- 性联鱼鳞病
英文词条(4)
- X-linked ichthyosis
- Sex-linked ichthyosis
- X-linked ichthyosis with steryl-sulfatase deficiency
- X-linked recessive ichthyosis
后组配轴 Postcoordination
该条目未定义后组配轴,直接使用上面的编码即可。后组配是在主干编码之后追加侧别、具体解剖部位、严重程度、病因等细节的可选组合方式,不是必填项。