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Dystrophic EB (DEB)

Dystrophic EB is characterised by a lack of adhesion of the skin under the basement membrane (found between the dermis and epidermis). Approximately 25% of all EB sufferers have dystrophic EB. The dystrophic type may be inherited as a dominant or a recessive trait.

The recessive inheritable types (RDEB) may lead to disability and, sometimes, a reduced life expectancy. However, there are many exceptions to this rule. Dystrophic EB derives its name from the tendency of the blisters to heal with scarring. This process can lead to contraction of the joints, fusion of the fingers and toes, contraction of the mouth membranes and narrowing of the oesophagus.

There is a wide variation in the severity of dystrophic EB. In general, dystrophic EB is not life-threatening in childhood. At its least severe (this is often the dominant inheritable type of dystrophic EB, DDEB) the patient can lead an almost normal life. However, the severity of the disorder does increase at a later age due to scarring, fusion of fingers and wastage of skin tissue. In the severe recessive type of dystrophic EB there is a high chance of developing a squamous cell carcinoma (skin cancer) before the age of 35. Unfortunately, this skin cancer is aggressive and life threatening.

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.