Epidermolysis Bullosa

  • Definition
  • Wound Care
  • Patient Advice and Prevention
  • Products
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Epidermolysis Bullosa is a rare genetic disease affecting ~500 000 people worldwide. It is the result of a failure of connection between the epidermis and the dermis.

 

 

 

To find out more

Epidermolysis Bullosa is characterised by the fragility of the epithelium leading to the formation of blisters at the slightest knock or rub.

On bursting, blisters are a cause of pain, open wounds and infection. The blistering can be confined to the hands and feet due to the friction, but in severest forms the whole body may be affected and the wounds heal slowly, giving rise to scarring, painful and weeping wounds.

There are more than 20 different forms of epidermolysis bullosa which can be divided into 3 groups according to the localisation of the symptoms: EB simplex, Dystrophic EB and Junctional EB.

To find out more visit http://www.epidermolysis-bullosa.com/

The wounds should be covered with non-adherent dressings such as Urgotul contact layer both to avoid pain and to encourage healing: In some areas, even with this type of dressing, it may be useful to apply a layer of vaseline.

The dressing should be covered with a sterile pad held in place by a very soft bandageand adhesive tape taking care that it does not adhere directly onto the skin and that the bandage is not too tight.

For wounds on the hands or feet, fingers and toes should be separated from each other to allow healing to take place: dressings should be inserted between the digits and held in place with a small band around the wrists and ankles.

 

To find out more, visit http://www.epidermolysis-bullosa.com/Learning-to-dress-wounds/Avoid-pain-and-sticking

 


These advices or recommendations do not replace expert opinion based on a full diagnosis.

Parents with new born baby diagnosed with EB

A new born baby with EB will probably stay in hospital for several weeks following diagnosis, to allow the parents enough time to learn how to look after them and to prepare for returning home.

Supporting links

It is a good idea to use this period for contacting the various agencies that can provide financial and practical aid.

Having a baby with EB can be stressful for parents however patient support and self-help groups exist throughout the world. For example visit: http://www.debra.org.uk/

Parents with child with EB

As the child grows parents will face new problems particularly with education and social interactions. In the majority of cases, children with EB are able to attend their local, main stream school.

However if the disease is too debilitating or preoccupying, it is possible to send children to a special school for people with motor disability, or to have them taught at home.

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To determine which solution is best, parents can consult the school of choice in advance and seek advice from their healthcare provider.

At school, activities are sometimes poorly suited to a child with EB. For example, physical education classes can be replaced by art, music, etc. However, in each case it is important to carefully consider the risks in relation to the benefits before taking a decision.

Having a child with EB may be stressful for parents. Support groups exist worldwide to help you e.g. http://www.debra.org.uk/

For more information see http://www.epidermolysis-bullosa.com/Daily-life/

Adults with EB

In cases of mild EB signs and symptoms may not develop until adolescence or early adulthood.

While no cure exists for epidermolysis bullosa the treatment should be followed:

  • Pain prevention
  • Wound care and prevention
  • Prevention of infection
  • Support programmes
  • Genetic counselling

These advices or recommendations do not replace expert opinion based on a full diagnosis.
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These advices or recommendations do not replace expert opinion based on a full diagnosis.
Last update : October 20, 2017