EC31
交界型大疱性表皮松解症
Junctional epidermolysis bullosa
- 上级条目
- 遗传性大疱性表皮松解症
- 层级
- 4 级
定义
交界性大疱性表皮松解症是由于表皮基底膜透明层水平的缺陷所致。全身严重型（Herlitz）与广泛内脏粘膜受累有关，婴儿期死亡率较高。
Junctional (lucidolytic) epidermolysis bullosa is due to defects at the level of the lamina lucida of the epidermal basement membrane. The generalised severe form (Herlitz) is associated with widespread mucosal involvement of internal organs and has a high mortality in infancy.
索引词 可用于检索该条目的同义词与别名
- 交界型大疱性表皮松解症
- 交界型EB[大疱性表皮松解症]
- JEB[交界型大疱性表皮松解症]
- 交界型大疱性表皮松解症
- 交界型大疱性表皮松解症，泛发型
- 交界型大疱性表皮松解症，重度泛发型
- Herlitz重型交界型大疱性表皮松解症
- JEB-H[交界型大疱性表皮松解症，Herlitz型]
- JEB-gen sev[交界型大疱性表皮松解症，重度泛发型]
- 致命大疱性表皮松解症
- Herlitz综合征
- 交界型大疱性表皮松解症，中度泛发型
- GABEB[泛发型萎缩性良性大疱性表皮松解症]
- JEB-nH gen[大疱性结膜表皮松解症，非Herlitz型，泛发型]
- JEB[交界型大疱性表皮松解症]非Herlitz型，泛发型
- 泛发型萎缩性良性大疱性表皮松解症
- JEB-gen intermed泛发型中度交界型大疱性表皮松解症]
- 交界性大疱性表皮松解伴幽门闭锁
- JEB[交界型大疱性表皮松解症]伴幽门闭锁
- JEB-PA[交界型大疱性表皮松解症伴幽门闭锁]
- 交界型大疱性表皮松解症，迟发型
- JEB-交界性大疱性表皮松解，迟发型
- JEB-LO[迟发型交界型大疱性表皮松解症]
- 进展性大疱性表皮松解
- 交界性大疱性表皮松解伴呼吸和肾脏受累
- 大疱性结合性表皮松解症，局限型
- 交界性大疱性表皮松解，局部型
- JEB-nH loc[交界型大疱性表皮松解症，非Herlitz型，局部型]
- JEB[交界型大疱性表皮松解症]非Herlitz型，局部型
- JEB-loc[交界型大疱性表皮松解症，局部型]
- 反常型交界型大疱性表皮松解症
- JEB [交界型大疱性表皮松解症]反常型
- JEB-inv[反常型交界型大疱性表皮松解症]
- 交界型大疱性表皮松解症，局部型
- JEB-LOC[交界型大疱性表皮松解喉甲皮肤]综合征
- LOC[喉甲皮肤]综合征
- LOGIC
- 喉-甲-皮肤综合征
- Shabbir综合征
英文词条（39）
- Junctional epidermolysis bullosa
- JEB - [junctional epidermolysis bullosa]
- Junctional EB - [epidermolysis bullosa]
- Lucidolytic epidermolysis bullosa
- Junctional epidermolysis bullosa, generalised types
- Junctional epidermolysis bullosa, generalised severe
- Epidermolysis bullosa letalis
- Herlitz junctional epidermolysis bullosa gravis
- Herlitz syndrome
- JEB-H - [junctional epidermolysis bullosa, Herlitz]
- JEB-gen sev - [junctional epidermolysis bullosa, generalised severe]
- Junctional epidermolysis bullosa, generalised intermediate type
- GABEB - [generalised atrophic benign epidermolysis bullosa]
- JEB - [junctional epidermolysis bullosa] non-Herlitz, generalised
- JEB-gen intermed - [junctional epidermolysis bullosa]
- JEB-nH gen - [junctional epidermolysis bullosa, non-Herlitz, generalised]
- generalised atrophic benign epidermolysis bullosa
- Junctional epidermolysis bullosa with pyloric atresia
- JEB - [junctional epidermolysis bullosa] with pyloric atresia
- JEB-PA - [junctional epidermolysis bullosa with pyloric atresia]
- Junctional epidermolysis bullosa, late onset
- Epidermolysis bullosa progressiva
- JEB - [junctional epidermolysis bullosa] late onset
- JEB-LO - [junctional epidermolysis bullosa late onset]
- Junctional epidermolysis bullosa with respiratory and renal involvement
- Junctional epidermolysis bullosa, localised types
- Junctional epidermolysis bullosa, localised
- JEB - [junctional epidermolysis bullosa] non-Herlitz, localised
- JEB-loc - [junctional epidermolysis bullosa, localised
- JEB-nH loc - [junctional epidermolysis bullosa, non-Herlitz, localised]
- Junctional epidermolysis bullosa inversa
- JEB - [junctional epidermolysis bullosa] inversa
- JEB-inv - [junctional epidermolysis bullosa inversa]
- Junctional epidermolysis bullosa, LOC
- JEB-LOC - [junctional epidermolysis bullosa laryngo-onycho-cutaneous] syndrome
- LOC - [laryngo-onycho-cutaneous] syndrome
- LOGIC
- Laryngo-onycho-cutaneous syndrome
- Shabbir syndrome
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。