Some facts about epidermolysis bullosa, or EB

Here are some facts about epidermolysis bullosa, or EB:

WHAT IS EB: A rare set of genetic disorders that cause blistering and shearing of the skin with even slight friction. There are three major types: simplex, dystrophic and junctional, which vary from mild to incapacitating and can sometimes be fatal.

BUTTERFLY CHILDREN: The name given to kids with ER because their skin is as fragile as a butterfly’s wings. They are also sometimes called “cotton wool babies.”

PREVALENCE: There are an estimated 3,500 to 5,000 Canadians with various types of the disease.

TREATMENT: There is no cure, but researchers are looking into possible treatments, such as stem-cell transplants and gene therapy, which are only experimental.

SKIN CANCER RISK: Patients with EB have a 70 per cent increased risk of developing squamous cell carcinoma (SCC) compared with the general population.

About 7.5 per cent of EB patients get the skin cancer by age 20, and about 80 per cent develop it by age 45.

An estimated 60 per cent will die by age 35 from SCC, which can be fast-moving and invasive in those with EB.

Online:

DEBRA Canada (Dystrophic Epidermolysis Bullosa Research Association): www.debracanada.org

DEBRA International: www.debra-international.org