About Epidermolysis Bullosa

What is Epidermolysis Bullosa

Epidermolysis Bullosa (EB) is an illness that causes the skin to be fragile or loose. Because the skin is so fragile, it can be easily injured, causing painful blisters to form. These blisters can cause serious problems if they become infected and this can be fatal.

EB is hereditary (runs in families).

Anyone can have EB. Generally, the symptoms first appear in babies or toddlers.

 

Clinical Types of EB


 

There are three main types of EB:

  • Simplex:
    Affects the epidermis (outer skin) all over the body.

  • Junctional:
    Affects the layer between the epidermis and dermis.

  • Dystrophic:
    Affects the dermis (inner skin) nearest the flesh.

 


 

What Are the Symptoms of Epidermolysis Bullosa?

Skin blisters are the major symptom of EB.

EB can be mild, moderate or severe and cause permanent scarring. Blisters can develop on the skin, the scalp causing hairloss, the nails resulting in nail loss, deformity of fingers and toes.

Thickening of palms and soles can also occur. Some blisters can be inside the body - in places such as the mouth, stomach, oesophagus (difficulty in swallowing), bladder and elsewhere.

There can also be dental abnormalities.

 

How Is Epidermolysis Bullosa Diagnosed?

Diagnosis is done by taking a small sample of skin and looking at it under a microscope.

 

How Is Epidermolysis Bullosa Treated?

There are doctors, nurses, social workers, psychologists, dietitians and local patient and parent support groups that can help.

 

 

 

Complications That May Arise

  • Secondary skin infection
  • Sepsis
  • Malnutrition
  • Anaemia
  • Eye disorders
  • Skin cancer
  • Hoarse voice
  • Dental complications