Epidermolysis bullosa 

Introduction 

Epidermolysis bullosa acquisita

Epidermolysis bullosa acquisita (EBA) is a separate condition to epidermolysis bullosa (EB) that has similar symptoms.

Like EB, EBA causes the skin to blister easily. It can also affect the mouth, throat and digestive tract.

However, unlike EB, EBA is not inherited. EBA is an autoimmune disease (a disease that affects your body's natural defence system) that usually doesn't appear until later life. It is not known exactly what causes it.

EBA is more common in people over the age of 40, but overall it is a very rare condition.

Epidermolysis bullosa (EB) is a general term used to describe a group of rare inherited skin disorders that cause the skin to become very fragile.

In people with EB, any trauma or friction to the skin can cause painful blisters.

Types of epidermolysis bullosa

There are three main types of EB, which are described below. The condition is classified according to where in the various layers of skin  blistering takes place.

  • Epidermolysis bullosa simplex (EBS), where blistering occurs in the upper layer of the skin (the epidermis). This is the most common type of EB, accounting for 70% of cases, and tends to be milder than the other types.
  • Junctional epidermolysis bullosa (JEB), where blistering occurs at the junction between the epidermis and the dermis (lower layer of the skin) in a layer of skin known as the basement membrane zone. JEB accounts for around 5% of cases and is usually considered the most severe type of EB.
  • Dystrophic epidermolysis bullosa (DEB), where blistering occurs below the basement membrane zone in the upper part of the dermis. DEB accounts for around 25% of cases.

There are many variants of these main types of EB, each with slightly different symptoms. So far, researchers have classified 27 variants of the condition and there may be more that have not yet been identified.

Read more about the symptoms of epidermolysis bullosa.

In most cases, symptoms of EB are obvious from birth or soon after. If it is suspected that your child has the condition, they will be referred to a skin specialist (dermatologist) who will carry out tests to determine the type of EB and will help to come up with a treatment plan.

Read more about diagnosing epidermolysis bullosa.

Why does EB happen?

EB is caused by faulty genes inherited from one or both parents.

In some cases, one or both parents will have the condition. However, there are many cases when neither parent has EB, but their child does. This happens when both parents are 'carriers' of the faulty genes without realising it.

If you or your partner is known to be a carrier of a mutated gene associated with EB and there is a risk of your child developing a severe form of the condition, it is possible to test an unborn baby at about 11 weeks into pregnancy. 

Read more about the causes of epidermolysis bullosa.

Who is affected?

EB is a rare condition. It is estimated that one in every 17,000 children born in the UK will have EB. In the UK, there are currently an estimated 5,000 people living with it.

EB affects both sexes equally.

How is EB treated?

There is currently no cure for EB, so treatment aims to relieve the symptoms and prevent complications developing, such as blisters becoming infected.

You will be helped by a team of medical specialists to decide what treatment is best for your child. They will also offer advice about living with the condition.

Most treatments can be done at home, such as lancing blisters with a sterile needle, and avoiding things that make the condition worse, such as heat and solid foods.

Medicines are only really used to treat infection or reduce pain. Surgery can be used if EB causes physical problems, such as narrowing of the airways due to scarring.

Read more about treating epidermolysis bullosa.

Outlook

The outlook for EB can vary widely depending on the type and variant of EB a child has.

Epidermolysis bullosa simplex (EBS) carries a low risk of causing serious complications. However, it can often be a distressing and frustrating condition to live with, as it can interfere with daily activities.

Some cases of dystrophic epidermolysis bullosa (DEB) are mild and cause no serious complications, while others are severe and can lead to skin cancer in later life.

The outlook for a very rare type of junctional epidermolysis bullosa (JEB) called Herlitz JEB is poor. As many as nine out of 10 children with this form of the condition will die from a serious complication developing during the first two years of life.

Page last reviewed: 30/10/2012

Next review due: 30/10/2014

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