Epidermolysis Bullosa Home Care Standard care plan patient information

Epidermolysis Bullosa, abbreviated to EB, is the name of a genetically-
determined skin disorder. In the Netherlands, around 400 patients have been recorded.

A characteristic feature of EB is the development of blisters on the skin after
slight pressure or rubbing. In healthy people, blistering only occurs after severe friction of the skin, for example during a long walk. EB-patients may develop blisters by simply wearing clothes and grabbing ordinary objects. In the course
of time, the patient may increasingly encounter restrictions because of the disease.

The blisters cause a lot of pain and discomfort. Especially when the blisters
are visible to others and alter the appearance, it means the social activities / contacts are limited. The blisters heal slowly and often leave scars.

EB presents itself in all kind of variations. There are about 27 different types known, varying from mild to severe. In the milder form of EB blistering only
occurs on hands and feet. The more serious forms are characterized by blisters
all over the body and even internally.

Internal blisters may develop in the mouth, the throat and the oesophagus.
These blisters complicate eating and drinking. This may result in a serious
delay in growth.

EB is a chronic illness. This means the disease cannot be cured.

As a result of thorough scientific research the cause of the various forms of EB has become more clear in recent years. Most forms of EB are hereditary, this means that the disease may be passed on to the next generation.

EB is not passed on by contagion.

Whether a child has inherited the disease or not, is usually obvious rather
quickly after the birth.

A distinction can be made between the dominant inheritable types and
recessive types of inheritance. A dominant form of inheritance means that
one of the parents is an EB-sufferer. The chance that the disease will be passed on to the next generation is 50% for each child.

In a recessive form of inheritance both parents are healthy, but a carrier of
the inheritable property that leads to EB. When two carriers join each child

has a 25% chance of having the disorder. Thousands of people are, without knowing, a carrier of a serious type of EB.

What does EB mean for the patient and his/her environment?

The disease EB may have a great effect on the patient and the environment. These effects are physical, such as blistering, wound infection, atrophic
scarring, itch, pain and tooth decay but there may also be psychosocial effects.