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    Your support of DEBRA International or a national EB group enables us to provide the best quality of life for families and individuals affected by EB.

Donation Account: IBAN AT65 6000 0005 1004 5254, BIC BAWAATWW, DEBRA International, Am Heumarkt 27/1, 1030 Vienna

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Junctional EB (JEB)

Junctional EB is characterised by lack of adhesion of the skin through the basement membrane (found between the dermis and epidermis). Approximately 5% of people with EB have the junctional form. Junctional EB is inherited recessively, i.e. the parents are carriers, often unknowingly, and do not show physical signs of the condition. The birth of a child with junctional EB is usually totally unexpected.

For some babies born with junctional EB the outlook is bleak; half of the children die within the first two years as the result of complications caused by serious blistering in the pharynx and the oesophagus. Characteristic clinical features include damage to and marking of the skin tissue on the face. These skin defects only develop after a couple of months. There are only a few patients with this severe type that survive the second or third year of life. The other half of children with JEB have a milder form which does not impose major life restrictions.

If you are reading this section because there is a new baby in your life with EB, please bear in mind that, while there is currently no safe and effective treatment for EB, there is much research on-going into possible treatments, some of which are showing promise. It is the hope of all in DEBRA International that over the life-time of babies born now, new treatments will emerge which will lessen the impact of EB.