Epidermolysis Bullosa (EB), is the name given to a group of genetic disorders that cause blistering and shearing to the skin from even the gentlest friction.
There are many strains of EB identified (over 20 at current count), each one with its own characteristics and symptoms.
The severity of the condition can range from relatively mild, through to completely incapacitating, and sometimes fatal.

The name is derived from the words:-

• Epidermis - outer skin layer
• Bullosa - blister
• Lysis - breakdown

The strains of EB can be grouped into three main categories: -

• EB Simplex
• Dystrophic EB
  • recessive
  • dominant
• Junctional EB

In very simple terms, EB causes blisters to form on the skin. These blisters may be restricted to specific area's, such as the hands or feet, or may effect a much larger area of the body.
In milder forms of the condition, these blisters heal up quite normally, without scaring or permanently damaging the skin.
In other form, the blisters do heal, but may leave scarring on the skin. This scarring can lead to the skin being permanently changed, for example the fingers may fuse together and hands may contract.

Some forms of Junctional EB are life threatening in infancy.

AT PRESENT, THERE IS NO KNOWN CURE

The strains of EB are all genetic in origin.
The defective genes result in the layers of skin not being able to bond together properly. This results in areas of structural weakness over the body. With the skin of an EB sufferer being so fragile, they are affectionately know as the Butterfly Children™.

Since EB is genetic in nature, its not contagious, nor is it infectious, so there is no risk to others from being in contact with someone who has the condition.

EB is hereditary.
Different forms of EB are passed down from parents to children in different ways.
In some cases the condition is passed directly from parent to child.
Other types of EB are transmitted when both parents carry the defective genes, however may not necessarily be EB sufferers themselves.
When this is the case, there is a chance that a child will inherit the condition form the parents, or just the defective genes, which means that although the child themselves is not affected, if they were to meet a partner who also carried the defective genes, the cycle could begin over again.

With advances in genetics, particularly the publication of the human genome, several of the genes which are responsible for certain sub-types of EB have been identified, although many others still remain elusive.

With the speed that medical science is advancing, researchers are optimistic about developing treatments for EB, and all hope that a cure will be discovered in the near future.

PLEASE REMEMBER

AT PRESENT, THERE IS NO KNOWN CURE

debra

Formed in 1978 by a group of parents, all with children who suffered with EB, the Dystrophic Epidermolysis Bullosa Research Association (DEBRA) aims to stimulate knowledge and interest in the condition, provide support to those affected by EB, their families and careers, and raise money to fund medical research in the hope of finding effective treatments and a cure.

DEBRA lists its own objectives as: -

1. To provide continuing services and support to people whose lives are affected by EB,
2. To develop effective treatments for EB through the funding of research into the condition.
3. To promote the best current practice in treatment.
4. To promote the wellbeing of people with EB and their families and assist in their full integration into Society.
5. To promote professional and public knowledge of EB.
6. To encourage the development of EB Support Groups around the world and to foster co-operation and liaison.
7. To raise sufficient funds to make achievement of these objectives possible.

Formed when only 200 or so sufferers were know to the medical profession, DEBRA now has independent branches in over 15 countries.

DEBRA also has an active medical research program aimed at developing treatments and an eventual cure.

Why I decided to put this page on my site.

Somebody very very close to me is affected by this condition, and before I met her I had never heard of it before.

The condition is rare (about 1 in 22,000 live births in the UK), and I wanted to do something to see if I could help raise the publics' awareness.

Obviously, what is presented here is only a very brief description of the condition. For more information about EB, and the organizations set up to support patients and their families, please follow one of the links below.

DEBRA America	DEBRA Australia	DEBRA Austria	DEBRA Belgium
DEBRA Ireland	DEBRA Netherlands	DEBRA New Zealand	DEBRA Sweden

DEBRA UK

DEBRA International

DEBRA Europe

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