vitalwiki

Severe generalized junctional epidermolysis bullosa

ORPHA:79404· ICD-10 Q81.1

Definition

A severe form of junctional epidermolysis bullosa (JEB) characterized by blisters and extensive erosions, localized to the skin and mucous membranes.

Prevalence
1-9 / 1 000 000
Inheritance
Autosomal recessive
Age of onset
Infancy, Neonatal