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Junctional EB

Junctional EB

Junctional EB (JEB) is characterised by a lack of adhesion of the skin through the basement membrane.

Approximately 10% of people living with EB have JEB. JEB is inherited recessively which means that the parents are both carriers, often unknowingly, and do not show physical signs of the skin condition. The birth of a child with JEB is therefore totally unexpected.

JEB generalised severe (previously Herlitz JEB), which affects around half of those diagnosed with JEB, is usually fatal in infancy with only a few babies surviving to the second or third year of life. These infants usually die from malnutrition and anaemia due to serious blistering in the pharynx and the oesophagus.

The milder form of JEB, JEB generalised intermediate (previously Non-Herlitz JEB), does not impose life restrictions, but can cause life-long pain and disability.

Alisa's story

Alisa's story

DEBRA-funded specialist EB nurses provided a lifeline to Alisa’s parents in the early days, showing them how to change her dressings, lance the blisters...a harrowing daily experience dreaded by both Alisa and her mum.

READ HER STORY