" /> Dystrophic epidermolysis bullosa - CISMeF





ICD-11 code : EC32;

Preferred Label : Dystrophic epidermolysis bullosa;

ICD-11 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.;

ICD-11 synonym : Dystrophic EB; Epidermolysis bullosa dystrophica; Dystrophic EB - [epidermolysis bullosa]; epidermolysis bullosa;

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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.

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31/05/2025


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