What is EB?

Epidermolysis Bullosa (EB) is a rare genetic disease. It is characterised by extremely fragile and blister prone skin. Management of the disease requires frequent application of specialised dressing and bandages to reduce skin damage and the risk of infection. The cost of dressings can be extremely high, particularly for those with more severe forms of EB.

There are many different forms of EB. The three main types are based on which level of skin is affected.

The main types are called:

EB Simplex – This is the most common form of EB. It is also generally the mildest form. The skin splits, forming blisters in the very top layers of the skin. There are various types of EB Simplex which can all look different.

Junctional EB – The layer of skin involved here is the middle layer. An abnormality here means that the blisters and erosions are deeper than in EB Simplex. Some different forms of this type are more severe than others.

Dystrophic EB – In this type of EB, the skin is fragile in the deepest layer. The term ‘dystrophic’ refers to the scarring that can occur following the healing of the blisters and erosions. The appearance also changes depending on type.

Definitions of EB types supplied courtesy of Royal Children’s Hospital Melbourne.

Within these categories, there are a number of sub-types based on inheritance and clinical features.

 

Accessing more information about EB

For more information about EB, including wound dressing education and links to national and international organisations, please click here.

You may also wish to contact DEBRA Australia, the Dystrophic Epidermolysis Bullosa Research Association of Australia