EC32
category
Dystrophic epidermolysis bullosa
Definition
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.
Also indexed as
Acral dystrophic epidermolysis bullosaAutosomal dominant dystrophic epidermolysis bullosaAutosomal recessive dystrophic epidermolysis bullosaBullous dermolysis of the newborn (recessive)DDEB pruriginosa - [dominant dystrophic epidermolysis bullosa pruriginosa]DDEB, generalised - [dominant dystrophic epidermolysis bullosa, generalised]DDEB, nails only - [dominant dystrophic epidermolysis bullosa, nails only]DDEB-BDN - [dominant bullous dermolysis of the newborn]DDEB-Pr - [dominant dystrophic epidermolysis bullosa pruriginosa]DDEB-Pt - [dominant dystrophic epidermolysis bullosa, pretibial]DDEB-ac - [dominant dystrophic epidermolysis bullosa, acral]DDEB-acral - [dominant dystrophic epidermolysis bullosa, acral]DDEB-gen - [dominant dystrophic epidermolysis bullosa, generalised]DDEB-na - [dominant dystrophic epidermolysis bullosa, nails only]DDEB-pretibial - [dominant dystrophic epidermolysis bullosa, pretibial]Dominant bullous dermolysis of the newbornDominant dystrophic epidermolysis bullosa pruriginosaDominant dystrophic epidermolysis bullosa, acralDominant dystrophic epidermolysis bullosa, generalisedDominant dystrophic epidermolysis bullosa, nails onlyDominant dystrophic epidermolysis bullosa, pretibialDystrophic EB - [epidermolysis bullosa]Dystrophic epidermolysis bullosaDystrophic epidermolysis bullosa, nails onlyEpidermolysis bullosa dystrophicaRDEB - [recessive dystrophic epidermolysis bullosa centripetalis] centripetalisRDEB - [recessive dystrophic epidermolysis bullosa] inversaRDEB - [recessive dystrophic epidermolysis bullosa] pretibialRDEB - [recessive dystrophic epidermolysis bullosa] pruriginosaRDEB Hallopeau-SiemensRDEB-BDN - [recessive bullous dermolysis of the newborn]RDEB-Ce - [recessive dystrophic epidermolysis bullosa centripetalis]RDEB-GS - [recessive dystrophic epidermolysis bullosa, generalised severe]RDEB-I - [recessive dystrophic epidermolysis bullosa inversa]RDEB-O - [recessive dystrophic epidermolysis bullosa, generalised other]RDEB-Pr - [recessive dystrophic epidermolysis bullosa pruriginosa]RDEB-Pt - [recessive dystrophic epidermolysis bullosa, pretibial]RDEB-gen intermed - [recessive dystrophic epidermolysis, generalised intermediate]RDEB-gen sev - [recessive dystrophic epidermolysis bullosa, generalised severe]RDEB-inv - [recessive dystrophic epidermolysis bullosa inversa]Recessive bullous dermolysis of the newbornRecessive dystrophic epidermolysis bullosa centripetalisRecessive dystrophic epidermolysis bullosa inversaRecessive dystrophic epidermolysis bullosa pruriginosaRecessive dystrophic epidermolysis bullosa, generalised intermediateRecessive dystrophic epidermolysis bullosa, generalised severeRecessive dystrophic epidermolysis bullosa, pretibialRecessive transient bullous dermolysis of the newborn