ICD-11 classes
14 Diseases of the skin
Genetic and developmental disorders affecting the skin
Genetically-determined epidermolysis bullosa
EC32 — Dystrophic epidermolysis bullosa

ICD-11 EC32 — Dystrophic epidermolysis bullosa

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.

The diagnosis includes nothing.

The diagnosis excludes nothing.

It has no clarifying diagnoses.