ICD-11 classes
14 Diseases of the skin
Genetic and developmental disorders affecting the skin
Genetically-determined epidermolysis bullosa
EC31 — Junctional epidermolysis bullosa

ICD-11 EC31 — Junctional epidermolysis bullosa

Junctional (lucidolytic) epidermolysis bullosa is due to defects at the level of the lamina lucida of the epidermal basement membrane. The generalised severe form (Herlitz) is associated with widespread mucosal involvement of internal organs and has a high mortality in infancy.

The diagnosis includes nothing.

The diagnosis excludes nothing.

It has no clarifying diagnoses.