EB

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 the blistering takes place.

Epidermolysis bullosa simplex (EBS) – the 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 it tends to be milder than the other types.

Junctional epidermolysis bullosa (JEB) – the 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) – the blistering occurs below the basement membrane zone in the upper part of the dermis. DEB accounts for around a quarter of cases.

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

How common is epidermolysis bullosa?

EB is a very 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. The symptoms usually begin at birth or shortly afterwards.

The faulty genes responsible for EB can be passed down through families. In some cases of EB, each person who has the affected gene shows signs of the condition. Other forms of EB are inherited differently: both parents may have the faulty gene but have no symptoms of EB and no previous history of the condition in their family. They can give birth to a child with EB if that child inherits the faulty gene from both parents.

Actiformcool wound dressing has successfully reduced pain and enouraged healing in patients with EB.