Thursday, 21 November 2013

JUNCTIONAL EB



Junctional EB - The blistering occurs at the lamina lucida level of the skin. Approximately 10% of people with EB will have JEB. All subtypes of JEB is inherited as a recessive disorder. With recessive traits and disorders, two copies of the gene are required in order for the trait or disorder to be present; therefore the parents do not have EB. They are called “carriers.”  In order for EB to occur in their children, both parents must pass the abnormal copy of the gene to child. There is a 25% chance (1:4), The birth of a child  with JEB is totally unexpected. Children that are born with JEB could die within the first two years.  The reason for that is malnutrition and anemia. This would be caused by blistering in the pharynx and the esophagus. There is about three types of JEB.

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