EPIDERMOLYSIS BULLOSA
SIMPLEX (EBS)
WHAT IS IT?
EB is a group of inherited disorders in which the skin blisters extremely easily. There
are 3 main types of EB. Each is a quite distinct disorder. If you have EB simplex then you
cannot develop one of the other forms of EB (dystrophic or junctional).
WHAT IS THE CAUSE?
The problem lies in the genes which hold the instructions necessary for production of
certain proteins in the top layer of skin. These instructions have a minor fault, rather
like a typing error, with the result that the proteins are incorrectly formed, and unable
to fulfil their role as scaffolding for the topmost layer of skin. The result is that the
top layer of skin does not “stick” securely to the layer beneath it, and where
the two layers separate a blister develops.
HOW DID YOU GET IT?
EBS is almost exclusively a dominantly inherited condition seen equally in males and
females. This means anyone who has EBS can pass the condition onto his or her children.
Each time a pregnancy occurs, there is a 1 in 2 chance that the child will inherit EBS if
one parent is affected. However, EBS can sometimes be seen as a ‘new mutation’
when there is no family history.
IS THERE A CURE?
Not yet – but research continues. There is still a long way to go, but an effective
treatment to prevent the blistering may ultimately be possible.
ARE THERE DIFFERENT TYPES OF EBS?
There are 3 main types of EBS –
Weber Cockayne EBS
Köbner EBS
and Dowling Meara EBS
This short information leaflet will describe the care and management of Weber Cockayne and
Köbner EBS.
DebRA produce a separate booklet pertaining to the management of Dowling Meara EBS.
WEBER COCKAYNE
This is the most common type of EBS. Blistering is localised to the hands and feet.
Blisters may not become evident until the child begins to walk.
KOBNER
Blistering may be obvious from birth, or develop during the first few weeks of life. Sites
of blistering respond to areas where friction is caused by clothing and frequently appear
around the edges of the nappy. Blisters are often seen inside the mouth but do not
generally cause a problem during feeding.
MANAGEMENT
Management involves identifying new blisters, lancing them with a sterile needle, or
snipping with a sharp pair of scissors to release the fluid and prevent spread. If
compressing the blister to expel the fluid is very painful, then the fluid can be
aspirated using a fine needle attached to a syringe. Many people find dressings
unnecessary, preferring to dust the area with cornflour to help dry up the blisters and
reduce friction. However, some non-stick dressings that could be tried are Aquacel and
Mepitel or Mepilex. These dressings can be held in place with a sock, cotton bandage or
tubular bandage such as Tubifast. Dressings are continually evolving. What one patient
likes another may not. The EB team can advise on up to date appropriate dressings.
PAIN RELIEF
Blisters can be very painful and limit mobility. Some people find it helpful to take
simple painkillers such as paracetamol and ibuprofen when their feet are especially sore.
Longer term pain management may be necessary, sometimes only in the summer months when
blistering is at its most troublesome. The DebRA EB medical team can offer advice on this
to your GP.
CONSTIPATION
Occasionally, a person can get a blister around the anal region (bottom), which can then
make it painful when passing a stool. This in turn leads to constipation, as a child is
frightened to open their bowels because it hurts. If this occurs, it is important not let
this continue. A good diet with plenty of fluids helps. Occasionally medicines to help
soften stools or to stimulate the bowel are prescribed.
PREVENTION
There are no measures that will totally stop all blisters forming, however the following
actions can be carried out to minimise the risk:
1. Heat, especially when associated with high humidity can exacerbate
blister formation, so measures taken to keep affected areas cool will often reduce the
rate of blistering.
Ø Wear natural materials
Ø Soak area (e.g. hands and feet) in cool water, especially after a hot
day
Ø Temperature regulation in house/classrooms
2. Choice of footwear is very important. As stated above natural materials for shoes are
good i.e. leather. Check the insides of new shoes for excessive seams, as these could
cause blisters. Children should get their feet measured to ensure good shoe fit. Some
recommended makes are Ricosta and Elefanten, both available from good shoe shops. Some
people find the use of insoles beneficial e.g. Carnation who also make Silversock, which
helps regulate foot temperature (telephone 0121 544 7117 for details). Walking some days
will not be a problem but some days can be difficult. Many people ‘save’
themselves to do activities that they enjoy.
WHAT HELP IS AVAILABLE
DebRA is the charity set up to help people with the condition EB. The contact number is
01344 771961. There website address is www.debra.org.uk.
They provide information, practical help, and professional help from nurses, social
workers, welfare workers and a means of communicating to families via newsletters and
conferences.
Some people with EBS have been successful in claiming DLA (Disability Living Allowance),
which can help towards extra costs involved in having EBS i.e. expensive footwear, bus
fares, taxi fares.
Some children have problems getting teachers and schools to understand their condition.
Literature is produced by DebRA to give to the school and the EB nurses can visit the
school if indicated.
November 2003
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