EC32
Dystrophic epidermolysis bullosa
Epidermólise bolhosa distrófica
CategoryDefinition
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.
Index Terms
Dystrophic epidermolysis bullosaEpidermolysis bullosa dystrophicaDystrophic EB - [epidermolysis bullosa]Autosomal dominant dystrophic epidermolysis bullosaDominant dystrophic epidermolysis bullosa, generalisedDDEB-gen - [dominant dystrophic epidermolysis bullosa, generalised]DDEB, generalised - [dominant dystrophic epidermolysis bullosa, generalised]Dominant dystrophic epidermolysis bullosa, acralAcral dystrophic epidermolysis bullosaDDEB-acral - [dominant dystrophic epidermolysis bullosa, acral]DDEB-ac - [dominant dystrophic epidermolysis bullosa, acral]Dominant dystrophic epidermolysis bullosa, pretibialDDEB-pretibial - [dominant dystrophic epidermolysis bullosa, pretibial]DDEB-Pt - [dominant dystrophic epidermolysis bullosa, pretibial]Dominant dystrophic epidermolysis bullosa pruriginosaDDEB pruriginosa - [dominant dystrophic epidermolysis bullosa pruriginosa]DDEB-Pr - [dominant dystrophic epidermolysis bullosa pruriginosa]Dominant dystrophic epidermolysis bullosa, nails onlyDystrophic epidermolysis bullosa, nails onlyDDEB, nails only - [dominant dystrophic epidermolysis bullosa, nails only]DDEB-na - [dominant dystrophic epidermolysis bullosa, nails only]Dominant bullous dermolysis of the newbornDDEB-BDN - [dominant bullous dermolysis of the newborn]Autosomal recessive dystrophic epidermolysis bullosaRecessive dystrophic epidermolysis bullosa, generalised severeRDEB Hallopeau-SiemensRDEB-GS - [recessive dystrophic epidermolysis bullosa, generalised severe]RDEB-gen sev - [recessive dystrophic epidermolysis bullosa, generalised severe]Recessive dystrophic epidermolysis bullosa, generalised intermediateRDEB-O - [recessive dystrophic epidermolysis bullosa, generalised other]RDEB-gen intermed - [recessive dystrophic epidermolysis, generalised intermediate]Recessive dystrophic epidermolysis bullosa inversaRDEB - [recessive dystrophic epidermolysis bullosa] inversaRDEB-I - [recessive dystrophic epidermolysis bullosa inversa]RDEB-inv - [recessive dystrophic epidermolysis bullosa inversa]Recessive dystrophic epidermolysis bullosa, pretibialRDEB - [recessive dystrophic epidermolysis bullosa] pretibialRDEB-Pt - [recessive dystrophic epidermolysis bullosa, pretibial]Recessive dystrophic epidermolysis bullosa pruriginosaRDEB - [recessive dystrophic epidermolysis bullosa] pruriginosaRDEB-Pr - [recessive dystrophic epidermolysis bullosa pruriginosa]Recessive dystrophic epidermolysis bullosa centripetalisRDEB - [recessive dystrophic epidermolysis bullosa centripetalis] centripetalisRDEB-Ce - [recessive dystrophic epidermolysis bullosa centripetalis]Recessive bullous dermolysis of the newbornBullous dermolysis of the newborn (recessive)Recessive transient bullous dermolysis of the newbornRDEB-BDN - [recessive bullous dermolysis of the newborn]