Catalog
Dystrophic epidermolysis bullosa
Link to WHO Definition
Dystrophic epidermolysis bullosa – http://id.who.int/icd/entity/1060981106
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.