EC32
营养不良型大疱性表皮松懈症
Dystrophic epidermolysis bullosa
- 上级条目
- 遗传性大疱性表皮松解症
- 层级
- 4 级
定义
营养不良（皮肤溶解性）大疱性表皮松解症是由于VII型胶原蛋白缺陷或缺失所致。因此，将表皮基底膜固定至真皮的锚定纤维存在缺陷或缺失。严重的隐性形式导致广泛的疤痕，易患侵袭性鳞状细胞癌。
Dystrophic (dermolytic) epidermolysis bullosa is due to defects in or absence of type VII collagen. As a result the anchoring fibrils which secure the epidermal basement membrane to the dermis are defective or absent. The severe recessive form causes extensive scarring and predisposes to aggressive squamous cell carcinoma.
索引词 可用于检索该条目的同义词与别名
- 营养不良型大疱性表皮松懈症
- 营养不良型大疱性表皮松懈症
- 营养不良型 EB[大疱性表皮松懈症]
- 常染色体显性遗传营养不良型大疱性表皮松解症
- 显性遗传营养不良型大疱性表皮松解症
- DDEB-Gen[显性遗传营养不良型大疱性表皮松解症，泛发型]
- DDEB，全身性[显性遗传营养不良型大疱性表皮松解症，泛发型]
- 显性遗传营养不良型大疱性表皮松解症，肢端
- Ddeb-Acral[显性遗传营养不良型大疱性表皮松解症，肢端]
- DDEB-ac[显性遗传营养不良型大疱性表皮松解症，肢端]
- 肢端营养不良型大疱性表皮松解症
- 显性遗传营养不良型大疱性表皮松解症，胫前
- DDEB-pretibal[显性遗传营养不良型大疱性表皮松解症，胫前]
- DDEB-PT[显性遗传营养不良型大疱性表皮松解症，胫前]
- 痒疹样显性遗传营养不良型大疱性表皮松解症
- DDeb Pruriginosa[痒疹样显性遗传营养不良型大疱性表皮松解症]
- DDeb-Pr[痒疹样显性遗传营养不良型大疱性表皮松解症]
- 显性遗传营养不良型大疱性表皮松解症，仅指甲
- DDEB，仅指甲[显性遗传营养不良型大疱性表皮松解症，仅指甲]
- DDEB-NA[显性遗传营养不良型大疱性表皮松解症，仅指甲]
- 营养不良型大疱性表皮松解症，仅指甲
- 新生儿显性遗传大疱性皮肤松解
- DDEB-BDN[新生儿大疱性皮肤松解症]
- 常染色体隐性遗传营养不良型大疱性表皮松解症
- 隐性遗传营养不良型大疱性表皮松解症，泛发重型
- RDEB-GS[隐性遗传营养不良型大疱性表皮松解症，泛发重型]
- RDEB-gen sev[隐性遗传营养不良型大疱性表皮松解症，泛发重型]
- 隐性营养不良型大疱性表皮松解症，Hallopeau-Siemens型
- 隐性遗传营养不良型大疱性表皮松解症，泛发中型
- RDEB-O[隐性遗传营养不良型大疱性表皮松解症，其他泛发型]
- RDeb-gen-intermed[隐性营养不良型表皮松解症，泛发中型]
- 反常型隐性遗传营养不良型大疱性表皮松解症
- RDEB[隐性遗传营养不良型大疱性表皮松解症]反常型
- RDEB-I[反常型隐性遗传营养不良型大疱性表皮松解症]
- RDEB-inv[反向型营养不良性大疱性表皮松解症]
- 隐性遗传营养不良型大疱性表皮松解症，胫前
- RDEB[隐性遗传营养不良型大疱性表皮松解症]胫前
- RDEB-Pt[隐性遗传营养不良型大疱性表皮松解症，胫前]
- 痒疹样隐性遗传营养不良型大疱性皮肤松解症
- RDEB[隐性遗传营养不良型大疱性表皮松解]痒疹型
- RDEB-Pr[痒疹样隐性遗传营养不良型大疱性皮肤松解症]
- 向心型隐性遗传营养不良型大疱性表皮松解症
- RDEB[向心型隐性遗传营养不良型大疱性表皮松解症]向心型
- RDEB CE[向心型隐性遗传营养不良型大疱性表皮松解症]
- 新生儿隐性遗传大疱性皮肤松解症
- RDEB-BDN[新生儿隐性遗传大疱性皮肤松解症]
- 新生儿隐性遗传暂时性大疱性皮肤松解症
- 新生儿大疱性皮肤松解症（隐性遗传）
英文词条（48）
- Dystrophic epidermolysis bullosa
- Dystrophic EB - [epidermolysis bullosa]
- Epidermolysis bullosa dystrophica
- Autosomal dominant dystrophic epidermolysis bullosa
- Dominant dystrophic epidermolysis bullosa, generalised
- DDEB, generalised - [dominant dystrophic epidermolysis bullosa, generalised]
- DDEB-gen - [dominant dystrophic epidermolysis bullosa, generalised]
- Dominant dystrophic epidermolysis bullosa, acral
- Acral dystrophic epidermolysis bullosa
- DDEB-ac - [dominant dystrophic epidermolysis bullosa, acral]
- DDEB-acral - [dominant dystrophic epidermolysis bullosa, acral]
- Dominant dystrophic epidermolysis bullosa, pretibial
- DDEB-Pt - [dominant dystrophic epidermolysis bullosa, pretibial]
- DDEB-pretibial - [dominant dystrophic epidermolysis bullosa, pretibial]
- Dominant dystrophic epidermolysis bullosa pruriginosa
- DDEB pruriginosa - [dominant dystrophic epidermolysis bullosa pruriginosa]
- DDEB-Pr - [dominant dystrophic epidermolysis bullosa pruriginosa]
- Dominant dystrophic epidermolysis bullosa, nails only
- DDEB, nails only - [dominant dystrophic epidermolysis bullosa, nails only]
- DDEB-na - [dominant dystrophic epidermolysis bullosa, nails only]
- Dystrophic epidermolysis bullosa, nails only
- Dominant bullous dermolysis of the newborn
- DDEB-BDN - [dominant bullous dermolysis of the newborn]
- Autosomal recessive dystrophic epidermolysis bullosa
- Recessive dystrophic epidermolysis bullosa, generalised severe
- RDEB Hallopeau-Siemens
- RDEB-GS - [recessive dystrophic epidermolysis bullosa, generalised severe]
- RDEB-gen sev - [recessive dystrophic epidermolysis bullosa, generalised severe]
- Recessive dystrophic epidermolysis bullosa, generalised intermediate
- RDEB-O - [recessive dystrophic epidermolysis bullosa, generalised other]
- RDEB-gen intermed - [recessive dystrophic epidermolysis, generalised intermediate]
- Recessive dystrophic epidermolysis bullosa inversa
- RDEB - [recessive dystrophic epidermolysis bullosa] inversa
- RDEB-I - [recessive dystrophic epidermolysis bullosa inversa]
- RDEB-inv - [recessive dystrophic epidermolysis bullosa inversa]
- Recessive dystrophic epidermolysis bullosa, pretibial
- RDEB - [recessive dystrophic epidermolysis bullosa] pretibial
- RDEB-Pt - [recessive dystrophic epidermolysis bullosa, pretibial]
- Recessive dystrophic epidermolysis bullosa pruriginosa
- RDEB - [recessive dystrophic epidermolysis bullosa] pruriginosa
- RDEB-Pr - [recessive dystrophic epidermolysis bullosa pruriginosa]
- Recessive dystrophic epidermolysis bullosa centripetalis
- RDEB - [recessive dystrophic epidermolysis bullosa centripetalis] centripetalis
- RDEB-Ce - [recessive dystrophic epidermolysis bullosa centripetalis]
- Recessive bullous dermolysis of the newborn
- Bullous dermolysis of the newborn (recessive)
- RDEB-BDN - [recessive bullous dermolysis of the newborn]
- Recessive transient bullous dermolysis of the newborn
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。