In this section
Epidermolysis bullosa (EB) is the name given to
a few genetic diseases that cause fragile skin. Because the skin is so
fragile (tender), it can be injured easily and cause blisters to
form. If these blisters become infected, they can cause serious
problems. Some people will have a few blisters while others will have a
lot. The blisters are most often found on the feet and hands but
can also form inside the mouth, the oesophagus (the tube that
allows food to go from the back of the mouth to the stomach), in
the stomach and the bladder. More severe EB can be found in
the teeth, nails, hair and other parts of the body.
Epidermolysis bullosa happens when one
of the important skin structures is decreased or totally missing.
The loss of this skin 'glue' makes the skin weak.
Currently, there is no
cure for EB. Many types are well controlled and many patients with
EB lead normal lives. Unfortunately, there are several rare types
that can cause death in infancy, or early adulthood.
EB is a rare disease, but
some types of it are more common than others. The symptoms usually
appear in babies and toddlers. Often there is a family history of
the condition, but this is not always the case. It is estimated
that less than 1 in 1 million children has some form of
There are many different
names for the different types of EB. The three main types are based
on which level of skin is affected. The 3 types are
This is the most common
form of EB. It is also the mildest form. The skin splits, forming
blisters in the very top layers of the skin. There are various
types of EB simplex that can all look different.
The level of skin involved
in junctional EB is the middle layer of the skin. An abnormality here means the
blisters and erosions are deeper than in EB simplex. Some forms of this type are more severe than others.
In this type of EB the skin
is fragile in the deepest layer. The term 'dystrophic' refers to
the scarring that can occur following healing of the blisters and
erosions. The appearance also changes depending the on the type.
If you get an infection, your doctor may:
Contact your doctor if your
Please note: These websites contain information
that may not be relevant to your child, depending on the type of
EB they have. Many websites are aimed at people with the most severe types of EB, and may be
upsetting to some people.
Developed in consultation with the RCH
Dermatology Dept. First published 2004. Updated November