Epidermolysis Bullosa

What is Epidermolysis Bullosa?

Epidermolysis bullosa is a group of rare diseases that cause fragile, blistering skin. The blisters may appear in response to minor injury, even from heat, rubbing, scratching or adhesive tape. In severe cases, the blisters may occur inside the body, such as the lining of the mouth or the stomach.

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 four 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.

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

Epidermolysis Bullosa Dystrophic: 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 (EB) is almost always caused by a genetic mutation that makes the skin extremely fragile. In rare conditions, it’s not inherited but is caused by the immune system.

Genetic causes of EB include the presence of a defective (or mutated) gene that is inherited from one (autosomal dominant inheritance) or both (autosomal recessive inheritance) parents, or it occurs as a spontaneous mutation, making the child the first in the family to have the disease.

Non-genetic reasons for EB a type that results from the immune system attacking the body’s healthy tissue by mistake.


Epidermolysis Bullosa signs and symptoms vary depending on type. They include:

  • Fragile skin that blisters easily, especially on the hands and feet
  • Nails that are thick or don’t form
  • Blisters inside the mouth and throat
  • Thickened skin on the palms and soles of the feet
  • Scalp blistering, scarring and hair loss (scarring alopecia)
  • Thin-appearing skin (atrophic scarring)
  • Tiny white skin bumps or pimples (milia)
  • Dental problems, such as tooth decay from poorly formed enamel
  • Difficulty swallowing (dysphagia)
  • Itchy, painful skin

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.


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.

  • Handle your child gently.Your infant or child needs cuddling, but be very gentle. To pick up your child, place him or her on soft material, such as cotton, and support under the buttocks and behind the neck. Don’t lift your child from under his or her arms.
  • Take special care with the diaper area.If your child wears diapers, remove the elastic bands and avoid cleansing wipes. Line the diaper with a nonstick dressing or spread it with a thick layer of zinc oxide paste.
  • Keep the home environment cool.Set your thermostat so that your home remains cool and the temperature remains steady.
  • Keep the skin moist.Gently apply lubricants, such as petroleum jelly.
  • Dress your child in soft clothes.Use soft clothing that’s simple to get on and off. It may help to remove labels and put clothing on seam-side out to minimize scratching. Try sewing foam pads into the lining of clothing by elbows, knees and other pressure points. Use soft special shoes, if possible.
  • Prevent scratching.Trim your child’s fingernails regularly. Consider putting mittens on him or her at bedtime to help prevent scratching and infection.
  • Encourage your child to be active.As your child grows, encourage him or her to be involved in activities that don’t cause skin injury. Swimming is a good option. For children with mild forms of epidermolysis bullosa, they can protect their skin by wearing long pants and sleeves for outdoor activities.
  • Cover hard surfaces. For example, place sheepskin on car seats and line the bathing tub with a thick towel.

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