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Epidermolysis bullosa: Diagnosis and treatment

If you or your child might have epidermolysis bullosa, it’s important to find out what’s going on. For anyone living with this condition, treatment is necessary to ease symptoms, protect their skin, and reduce the risk of developing complications like an infection.

The following explains how this condition is diagnosed and what treatment may involve.

How do dermatologists diagnose epidermolysis bullosa?

Getting a diagnosis takes time. Your dermatologist begins by closely examining the skin and other parts of the body that this condition can affect, like the inside of the mouth.

Your dermatologist will want to know if any blood relatives have/had epidermolysis bullosa or a condition that causes blisters to form

This information can be helpful, so try to find out before seeing your dermatologist.

Parent asking family member if any blood relative has or had epidermolysis bullosa

If your dermatologist suspects epidermolysis bullosa after examining the skin and learning about blood relatives, specialized testing is required. This is the only way to know for certain whether someone has this condition.

Your dermatologist may also recommend genetic testing. It can identify the genes causing epidermolysis bullosa.

After gathering all the information and test results, your dermatologist will give you the diagnosis. If the diagnosis is epidermolysis bullosa, your dermatologist will talk with you about treatment.

How is epidermolysis bullosa treated?

People diagnosed with epidermolysis bullosa are often referred to a specialized treatment center when possible. At these centers, you’ll find dermatologists, pediatric dermatologists, nurses, dieticians, psychologists, and others who have expertise in managing this rare condition.

If you cannot go to a specialized treatment center, you will see a doctor who has experience treating patients who have this condition, like a pediatric dermatologist or dermatologist. This doctor will be your primary doctor for epidermolysis bullosa, and you’ll be referred to other specialists as needed.

The doctor (or team) caring for a patient with epidermolysis bullosa develops a treatment plan carefully tailored to the patient’s needs. This care plan is written and given to the family and others who will care for the patient.

The amount of care that someone needs will vary. Parents who have a child with mild epidermolysis bullosa may be able to care for the child without much help once they learn how to treat the wounds and prevent new injuries.

When the condition is moderate or severe, the patient requires professional medical help, often from a team of medical professionals with different areas of expertise. A nurse may go to the home of a patient to help with treatment.

A treatment plan usually involves the following:

Wound care: Dressings, bandages, and sometimes medications are used to help skin heal. Dressings are placed directly on the wound to absorb blood and other fluids. Bandages hold the dressings in place.

Wound care involves changing bandages and dressings, usually daily

When treating wounds, wearing a new pair of clean medical gloves each time can help prevent an infection from developing in a wound.

Parent placing bandage over dressing for a child with epidermolysis bullosa

Most bandages and dressings are changed daily. However, some wounds may be treated every other day due to the discomfort caused by removing the dressings.

Great care is taken when removing bandages and dressings, which are usually changed one at a time. To remove bandages, some patients soak their wounds for 5 to 10 minutes. This may be done in a bathtub that’s filled with water and a small amount of vinegar or bleach. Once a dressing is removed, the person caring for the wounds will look for signs of infection or inflammation, as these can interfere with wound healing.

Each wound is then gently cleansed with a mild, non-toxic solution like saline, treated with medication if necessary, and covered with a new dressing and bandage.

The U.S. Food and Drug Administration (FDA) has approved two medications specifically for treating the wounds:

  • Gene therapy applied to wounds (beremagene geperpavec-svdt): This medication can accelerate wound healing in people with a type of epidermolysis bullosa called dystrophic epidermolysis bullosa (DEB).

    DEB impairs the body’s ability to produce a type of collagen that holds the layers of skin together. This medication, which is applied to the wound, contains a gene that can restore the skin’s ability to make the needed collagen.

    In a clinical trial of 31 patients with DEB, who had either the recessive (RDEB) or dominant type (DDEB) of DEB, each patient had two wounds of about the same size treated. One wound was treated with this medication, and the other was treated with what looked like the medication but contained no medication.

    After 26 weeks, 65% of the wounds treated with the medication had completely closed. Only 26% of the wounds that didn’t receive medication had closed.

    Side effects can occur. In the clinical trial, the most common side effects included itching, chills, rash, cough, and a runny nose. None of the patients stopped treatment due to side effects.

    Only a medical professional like a dermatologist or nurse should apply this medication, which is given once a week. Your dermatologist or nurse will decide which wounds to treat. Treatment continues until a wound heals completely.

    Anyone who is pregnant should avoid direct contact with this medication, the treated wounds, and dressings used on the treated wounds.

    The FDA approved this medication to treat patients with DEB who are 6 months of age and older. It offers hope because it may reduce or prevent non-healing wounds, infections, and discomfort.

    • Brand name: Vyjuvek

  • Gel containing birch bark (B-VEC): This medication is approved to treat open wounds that may not heal in people who have either dystrophic epidermolysis bullosa (DEB) or junctional epidermolysis bullosa (JEB).

    The gel contains extracts from the bark of birch trees that have naturally occurring substances called triterpenes.

    The FDA approved this medication after reviewing the results from a clinical trial of 233 patients (156 were children) in 28 countries. In this trial, two wounds of about the same size were treated on each patient. One wound was treated with B-VEC gel, and the other was treated with a gel that contained no medication.

    Within 45 days, 41% of patients had healing of the wounds treated with B-VEC gel.

    The most common side effects were itchiness and pain where the medication was applied.

    This medication can be applied at home to the wounds, which are then covered with a dressing. B-VEC can be used daily until the wound heals.

    • Brand name: Filsuvez

Pain management: Pain is a common symptom.Many things can cause pain, including the blisters and raw skin, dressing changes, an infection, swallowing, eating, or just moving.

Medication is often needed to relieve the pain. Antidepressants, medication used to treat epilepsy, or the pain reliever acetaminophen can help. If the pain is severe, fentanyl, morphine, or ketamine can be prescribed.

Before bathing and wound care, it may be necessary to give pain medication. Adding salt to the bathwater and using dressings that contain pain medication can also help reduce pain during wound care.

Treat itchiness: Itch is another common symptom. Itch can be triggered by inflammation, overheating due to wearing bandages, or even a pain medication.

To treat itch, your dermatologist will start by asking questions to find out what could be the cause. Treatment may include using different bandages or taking an antihistamine.

Prevent new injuries: When someone has epidermolysis bullosa, their skin is easily wounded. To prevent new wounds, your dermatologist may recommend using padded bandages or wearing loose-fitting clothes. Tight clothing can rub on the skin, causing it to slide off.

Your dermatologist can give you detailed advice that can help avoid new injuries.

Watch for and treat complications: Sometimes, blisters and wounds lead to other health problems, which your dermatologist may call complications. Here are possible complications that can occur and what treatment may involve:

  • Anemia: Good wound care, along with preventing and treating an infection, can reduce the blood loss that leads to anemia. When anemia develops, your dermatologist may treat it with iron supplements, an injection of iron, or a blood transfusion.

  • Cavities and other problems in the mouth: Gum disease and cavities are common. It’s helpful to see a dentist who has experience treating people who have epidermolysis bullosa. Most patients see their dentist every 3 months.

  • Constipation: Epidermolysis bullosa can make bowel movements painful, so some patients become constipated. A dietitian may be able to prevent constipation and other problems like diarrhea by changing the patient’s diet.

  • Dehydration: Open wounds can lead to dehydration. This can be treated by giving the patient lots of fluids to drink. Sometimes, an IV is needed to replace lost fluids.

  • Dry skin: Applying moisturizer as recommended by your dermatologist can help reduce dry skin, which can reduce blistering, pain, and itching.

  • Excess sweating: Common in patients with a type of epidermolysis bullosa known as epidermolysis bullosa simplex, excessive sweating should be treated. Treating it can reduce blistering and itching. Your dermatologist may treat the excess sweating with an antiperspirant. If stronger treatment is required, an antibiotic, antihistamine, or injections of botulinum toxin may be effective.

  • Eye problems: Sores on the eye, blistering eyelids, and other eye problems can occur. The sooner these are treated, the better. Some eye problems can be treated with eye drops used to lubricate dry eyes. Your dermatologist may also refer you to an ophthalmologist (eye doctor).

  • Fingers or toes join together: As skin heals on the fingers or toes, the digits can join together, becoming one. Special dressings can help keep them apart. Surgery can separate fingers or toes that have joined.

  • Infection: This can often be prevented if everyone treating the wounds has clean hands. Good wound care and proper nutrition can also help. Even with these precautions, an infection can occur. Treatment often involves changing dressings and bandages daily, along with using an antibiotic. A severe infection can last a long time.

  • Malnutrition: The body may use most of the nutrition it gets to heal wounds, leaving nothing for the rest of the body. This can prevent a child from growing. Malnutrition also occurs when blisters form in the mouth or esophagus, making eating painful. A dietitian may be able to recommend foods to eat. Sometimes, a feeding tube is needed for a while.

  • Narrowing of the mouth, esophagus, or urethra: Scarring can cause these parts of the body to narrow. Surgery can open them.

  • Non-healing wounds: A special wound covering can help. Some chronic wounds, however, will not fully close. FDA-approved medications can also help heal some wounds.

  • Problems moving: Physical and occupational therapy can prevent (or delay) shortening of the muscles and tendons, which can lead to deformities. Hydrotherapy (exercises done in water) can be especially helpful.

  • Skin cancer: This can develop in areas with long-standing wounds and scars and begin in a patient’s 20s or 30s. Surgery is often the most effective way to remove skin cancer. Sometimes, other types of treatment for skin cancer are recommended.

Research improving treatment

The FDA has approved two medications that can help heal wounds. Researchers continue to investigate other treatment options. These treatments focus on preventing epidermolysis bullosa from worsening and giving patients the genes needed to restore their skin.

What is the outlook for someone who has epidermolysis bullosa?

If a child has mild epidermolysis bullosa, the condition may become less severe with age. Life as an adult may not require time-consuming wound care or pain medication.

If the condition is moderate or severe, a person can require hours of care each day for life. A nurse’s help may be necessary to bathe and change dressings and bandages. The patient may frequently see medical doctors from different specialties including dermatology, ophthalmology, gastroenterology, and hematology.

Severe epidermolysis bullosa can be life-threatening due to widespread blistering, infections, and loss of body fluid.

While these generalizations apply, it’s important to remember that each person who has epidermolysis bullosa is different. Generalizations cannot predict what will happen to one person.

Parents and other caregivers can help make life more comfortable for someone with epidermolysis bullosa by following these dermatologists’ tips.

Getty Images


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Written by:
Paula Ludmann, MS

Reviewed by:
Brendan Camp, MD, FAAD
Carla Torres-Zegarra, MD, FAAD
Ata Moshiri, MD, FAAD

Last updated: 6/7/24