EC31
category
Junctional epidermolysis bullosa
Definition
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.
Also indexed as
Epidermolysis bullosa letalisEpidermolysis bullosa progressivaGABEB - [generalised atrophic benign epidermolysis bullosa]Herlitz junctional epidermolysis bullosa gravisHerlitz syndromeJEB - [junctional epidermolysis bullosa]JEB - [junctional epidermolysis bullosa] inversaJEB - [junctional epidermolysis bullosa] late onsetJEB - [junctional epidermolysis bullosa] non-Herlitz, generalisedJEB - [junctional epidermolysis bullosa] non-Herlitz, localisedJEB - [junctional epidermolysis bullosa] with pyloric atresiaJEB-H - [junctional epidermolysis bullosa, Herlitz]JEB-LO - [junctional epidermolysis bullosa late onset]JEB-LOC - [junctional epidermolysis bullosa laryngo-onycho-cutaneous] syndromeJEB-PA - [junctional epidermolysis bullosa with pyloric atresia]JEB-gen intermed - [junctional epidermolysis bullosa]JEB-gen sev - [junctional epidermolysis bullosa, generalised severe]JEB-inv - [junctional epidermolysis bullosa inversa]JEB-loc - [junctional epidermolysis bullosa, localisedJEB-nH gen - [junctional epidermolysis bullosa, non-Herlitz, generalised]JEB-nH loc - [junctional epidermolysis bullosa, non-Herlitz, localised]Junctional EB - [epidermolysis bullosa]Junctional epidermolysis bullosaJunctional epidermolysis bullosa inversaJunctional epidermolysis bullosa with pyloric atresiaJunctional epidermolysis bullosa with respiratory and renal involvementJunctional epidermolysis bullosa, LOCJunctional epidermolysis bullosa, generalised intermediate typeJunctional epidermolysis bullosa, generalised severeJunctional epidermolysis bullosa, generalised typesJunctional epidermolysis bullosa, late onsetJunctional epidermolysis bullosa, localisedJunctional epidermolysis bullosa, localised typesLOC - [laryngo-onycho-cutaneous] syndromeLOGICLaryngo-onycho-cutaneous syndromeLucidolytic epidermolysis bullosaShabbir syndromegeneralised atrophic benign epidermolysis bullosa