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.
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.
Almost all types of epidermolysis bullosa run in families. If you inherited certain gene glitches from your parents, you will have it.
There’s one exception. Epidermolysis bullosa aquisita is the only type that’s not inherited. It happens because of a problem in your immune system.
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.
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.
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.
Tips for At-Home Care
To help prevent blisters, you have to take special care of your skin.
Reduce friction. Use lotion to keep your skin moist and reduce friction. If you cover wounds, use only dressings that aren’t sticky, then loosely wrap again with rolled gauze. Wear loose-fitting clothes without tags, tight sleeves, or seams.
Drain blisters. If left untreated, these can fill with fluid and become infected. Your doctor can show you the best way to drain them.
Keep cool. Keep your bath water no warmer than room temperature. Stay in air conditioning as much as you can and avoid heat and humidity.
Know the signs of infection. Your skin may get red or feel hot to the touch if it is becoming infected. You could also notice pus or a yellow discharge crusting at the site, a red streak under the skin, or have fever or chills. If you see any of these signs, call your doctor right away. You may need antibiotics.
Check your diet. Many people with epidermolysis bullosa have low levels of iron, selenium, or vitamin D. Your doctor may suggest that you see a nutritionist about eating more foods that are rich in these vitamins and minerals.
Get support. Talk to your doctor or someone you trust. You might also find it helpful to talk about your feelings to a therapist or at a support group in your area.