What Is Epidermolysis Bullosa?

Epidermolysis bullosa is a rare genetic condition that makes skin so fragile that it can tear or blister at the slightest touch. Children born with it are often called “Butterfly Children” because their skin seems as fragile as a butterfly wing.

Mild forms may get better with time. But severe cases can be painful, trigger other serious health issues, and can be life-threatening.

If you have this condition, you need special treatment to keep your delicate skin as healthy as possible.

Types

There are five major types of epidermolysis bullosa. The kind you have depends on where your blisters tend to form.

Epidermolysis Bullosa Simplex: The most common type, it first shows up in newborns. It mainly affects the palms of the hands and soles of the feet.

Junctional epidermolysis bullosa: While it also first appears in babies, this is a more severe form that causes blistering in deep layers of the skin.

Dystrophic epidermolysis bullosa: If you have this type, your skin doesn’t have collagen to hold it together, or the collagen you do have doesn’t work well. This means the layers of your skin don’t seal together like they should. Sometimes this type doesn’t show up until early childhood.

Kindler syndrome: This is a mixed condition, since blisters appear across different skin layers. It can also cause patchy changes in your skin coloring when it’s exposed to the sun.

Epidermolysis bullosa acquisita: This form causes blisters on your hands and feet as well as in mucous membranes like the mouth.

Symptoms

Usually, signs of epidermolysis bullosa first appear in babies or toddlers. Painful skin blisters are the main symptom. They can form anywhere on the skin. Sometimes they also form on the eyes, or in parts of the throat, stomach, or bladder. If these blisters become infected or scar the skin, they cause more problems.

Treatment

There’s no cure for epidermolysis bullosa. But there are treatments for it.

If you have a severe case, you’ll care for your skin much like someone who has a burn does. You’ll need to learn how to perform daily wound treatment and how to bandage and protect affected areas.

Your doctor can also prescribe a medicine to help with pain relief.

In some cases, you might need surgery. If you have blisters that have fused your fingers and toes together, your doctor can separate them. Or if your esophagus, the tube connecting your mouth to your stomach, becomes too scarred, you can get surgery to widen it to help you eat.

Some people with epidermolysis bullosa still find eating too painful. In that case, your doctor may suggest a feeding tube so food goes right into your stomach.

Diagnosis

To confirm the condition, your doctor may take a small sample of skin and send it to a lab where experts will use a microscope to look at it.