Epidermolysis bullosa (EB) is a group of rare genetic skin conditions that cause the skin to blister. Genetic conditions are inherited, which mean that they are caused by faulty genes that run in the family.
Types of EB
There are three main types of EB:
- EB simplex. This is the most common type of EB and affects about 70% of people with EB in the UK.
- Dystrophic EB. This affects about 25% of people with EB.
- Junctional EB. This affects about 5% of people with EB.
Within each type of EB, there are several different forms, as symptoms and causes can vary. Currently, there are more than 25 known forms of EB.
Experts in EB recommended in 2008 that Kindler syndrome be included within the EB group. Kindler syndrome is a rare, genetic skin condition with similar symptoms. Since the condition was first identified in 1954, around 100 cases have been recorded.
See the Symptoms and Causes sections (above) for more information about the different types of EB.
Who is affected by EB?
EB mainly affects babies and children. The symptoms are usually present at birth or develop soon after. Children with EB are sometimes called butterfly children because their skin is said to be as fragile as a butterfly's wing.
EB affects boys and girls equally, and can affect any ethnic group. In rare cases, EB can also affect adults. In adults, the symptoms develop later in life and the condition tends to be mild.
About 5,000 people in the UK are thought to have EB.
Blisters and fragile skin
If a child has EB, faulty genes in their skin cells cause blisters to form. Their skin is also easily damaged (fragile). Even the slightest rubbing (friction) or minor injury can cause their skin to blister and tear.
Blisters can also develop on their own, even if the skin is not damaged. Blisters and skin damage can leave open wounds and raw patches on the skin, which can be very painful.
In some forms of EB, this can make everyday activities, such as learning to crawl and walk, difficult for both babies and older children.
EB can also cause problems with feeding your baby, or with eating in older children. You may need to take extra care when you touch your child, for example, when you pick them up.
EB can be mild or severe
Some forms of EB can be mild. Symptoms may affect only part of the body (localised), such as the feet and hands. Symptoms of some forms can also improve as a child grows older.
In other forms of EB, blisters and skin damage can be more widespread. Sometimes, complications can affect other parts of the body. Blisters may form in the mouth, eyes, nose, stomach, airways and genitals.
Some forms of EB can be severe, and the condition can be life threatening.
EB acquisita is a rare skin condition that mainly affects adults. Like of other forms of EB, EB acquisita causes blistering. However, EB acquisita is not a genetic disease; it is an autoimmune condition.
The immune system produces antibodies, which normally attack foreign cells such as bacteria. In an autoimmune condition, the antibodies attack the body's own healthy cells and tissue. In EB acquisita, antibodies attack and destroy skin cells, causing blisters to form.
EB acquisita is sometimes linked with other conditions such as:
Genetic is a term that refers to genes: the characteristics inherited from a family member.