Group of inherited disorders with blister formation in response to mechanical trauma
Aetiology
10 genes involved in skin structure and adhesion
Pathophysiology
Three main types - simplex, junctional, dystrophic
Clinical features
Epidermolysis bullosa simplex usually has little or no extracutaneous involvement, while the more severe hemidesmosomal, junctional and dystrophic forms of EB may produce significant multiorgan system involvement