Epidermolysis bullosa

  • Overview

    Epidermolysis bullosa (EB): Overview

    Epidermolysis bullosa causes blisters, which quickly burst and leave slow-healing wounds like the one on this baby’s knee.

    What exactly is EB?

    Epidermolysis bullosa (ep-ē-der-mol-eh-sis bah-low-sah) is a group of rare diseases that cause the skin to blister easily.

    The skin blisters because it’s so fragile. The fragile skin is usually noticeable at birth. A hug, loving rub on the back, or diaper change can injure the skin, leading to blisters and open wounds.

    Some children develop blisters in the moist tissue that lines the mouth, throat, stomach, intestines, rectum, and other areas of the body. In these areas, the friction caused by swallowing food or having a bowel movement can lead to painful blisters.

    The effects that EB has on a child’s life varies considerably. Some children have a mild disease that requires taking precautions to prevent injuries. At the other end of the spectrum, EB can cause lifelong disability that needs ongoing medical care.

    Some types of EB are less likely to cause lifelong disability, but any type of EB can cause severe symptoms, as the following descriptions of the different types of EB indicate.

    Types of epidermolysis bullosa (EB)

    Epidermolysis bullosa simplex (EBS): This type of EB causes blistering in the outermost layer of skin, which is the epidermis.

    Epidermolysis bullosa simplex (EBS): This is the most common type. In its mildest form, the blisters usually occur only on the hands and feet of a newborn. Later in life, the skin may stop blistering, leaving a teen or adult with thickened, hard skin on the palms and soles.

    EBS can also be more severe. Blistering may be widespread and occur anywhere on the skin or inside the mouth. The nails can be rough and thick or missing. When this happens, the blistering tends to be more serious at birth, but it still lessens in severity with age.

    As the blisters clear, the skin tends to heal without scarring.

    Dystrophic epidermolysis bullosa (DEB): When a child has a mild case of DEB, blisters tends to develop only on the hands, feet, knees, and elbows.

    Dystrophic epidermolysis bullosa (DEB): Blisters form in the middle layer of skin, which is the dermis, so scarring occurs as the blisters clear.

    Blistering can also be widespread, causing severe DEB. A baby born with severe DEB, can have widespread blistering and areas of missing skin. Blisters may develop inside the mouth and esophagus, which makes swallowing painful. When this happens, the child may need a feeding tube.

    As the blisters heal, scars tend to form. When skin on the fingers or toes repeatedly blisters and scars, the fingers or toes can join together. Repeated blistering and scarring is what caused the child shown in this picture to have fused toes. When the toes join together, walking becomes difficult.

    If your child’s fingers or toes repeatedly blister and scar, precautions can be taken to prevent them from joining together.

    People with DEB can also develop fatal skin cancers. This happens because the skin cancer is much more aggressive, which makes it difficult to treat the cancer successfully.

    You can find out what it’s like to live with a severe type of DEB by reading Fragile skin, strong spirit: Jonathan Gionfriddo’s story.

    Junctional epidermolysis bullosa (JEB): Blisters form in an area of the skin that lies between the outer and middle layers of skin, which is called the basement membrane.
    Junctional epidermolysis bullosa (JEB): When JEB is mild, blisters usually develop only on the newborn’s hands, elbows, knees, and feet — and the blistering tends to lessen by 2 or 3 months of age.


    JEB can also be severe, with blisters developing on large areas of the body or digestive tract.

    As blisters heal, scars appear. This can cause the toes or fingers to join together, but taking precautions can reduce the risk of this happening.

    Infections, hair loss, and breathing problems can also occur. When very severe, a child with JEB often dies during the first year of life.

    Kindler syndrome: This is a very rare type of EB. Only about 100 cases of Kindler syndrome have been reported since 1954. The largest number of known cases occurred within a tribe that lives in rural Panama.

    Kindler syndrome: Blistering lessens as a child grows, and dark spots appear on the skin.

    At birth, the skin blisters easily and is extremely sensitive to the sun. As the child grows, the blistering and sensitivity to the sun will lessen. As these diminish, a skin condition called poikiloderma (poy-key-low-der-ma) appears. It causes dark patches on the skin, so the skin starts to look a lot like that shown in this picture.

    At first, the dark patches appear only on skin that gets some sun. After puberty, the dark patches tend to appear everywhere on the skin. By this stage, you may also see visible blood vessels and the skin on the hands and feet tends to thin greatly. The skin also becomes so dry and wrinkled that the hands and feet resemble that of an elderly person’s.

    Epidermolysis bullosa acquisita: As the fluid-filled blisters (arrow) heal, you may see scars or small white bumps called milia (left and right).
    Epidermolysis bullosa acquisita (EBA): Extremely rare, this type of EB differs from the other types. This type of EB is thought to be an autoimmune disease. When someone develops an autoimmune disease, the person’s immune system mistakes a part of the body as foreign and attacks it.


    When a person has EBA, the body attacks the body’s collagen, which is found in the skin and throughout the body. This attack leads to blistering.

    Unlike the other types of EB, which begin at birth or shortly thereafter, EBA usually begins in one’s 30s or 40s. Blisters tend to develop on the hands, knees, elbows, and ankles. Blisters can also be widespread and form inside the mouth, nose, or eyes.

    As the blisters heal, white bumps called milia (mill-ē-ah) may appear. Sometimes, scarring develops. Blisters inside the body tend to cause severe scarring.

    Researchers are making progress in the treatment of EB

    While EB cannot be cured, researchers are making strides in understanding this rare group of diseases. This understanding has led to new treatments that can provide better relief from the symptoms.

    You’ll find more information about possible symptoms at Epidermolysis bullosa: Signs and symptoms.

    Images used with permission of the American Academy of Dermatology National Library of Dermatologic Teaching Slides.

    Fine, JD. “Epidermolysis bullosa.” In: Bolognia JL, et al. Dermatology. (second edition). Mosby Elsevier, Spain, 2008:457-66.

    Fine JD, Bruckner-Tuderman L, et al. “Inherited epidermolysis bullosa: Updated recommendations on diagnosis and classification.” J Am Acad Dermatol. 2014;70:1103-26.

    Ghosh SK, Bandyopadhyay D, et al. “Kindler’s Syndrome: A case series of three Indian children.” Indian J Dermatol. 2010;55:393-6.

    Hilton L. “EB’s great hope: Pediatricians respond to the first successful gene therapy treatment.” Dermatol Times. 2018;39(2):1+.

    Penagos H, Jaen M, et al. “Kindler syndrome in native Americans from Panama: Report of 26 cases.” Arch Dermatol. 2004;140:939-44.

    Seta V, Aucouturier F, et al. “Comparison of 3 type VII collagen (C7) assays for serologic diagnosis of epidermolysis bullosa acquisita (EBA).” J Am Acad Dermatol. 2016;74:1166-72.

    Watkins, J. “Diagnosis, treatment and management of epidermolysis bullosa.” Br J Nurs. 2016 Apr 28;25(8):428-31.

    Epidermolysis bullosa
  • Symptoms

    Epidermolysis bullosa (EB): Signs and symptoms

    With most types of epidermolysis bullosa (EB), signs are usually noticeable at birth or soon afterward. If a child has mild EB, however, the signs may appear when the child starts to crawl or walk.

    Regardless of when EB begins, you will notice:

    Blisters that rupture, causing a wound

    Everyone with EB has extremely fragile skin. Sometimes, the skin is so fragile that a gentle touch or sudden temperature change can cause painful blisters within minutes. As the blisters rupture, a wound appears.

    Children born with EB are often called “butterfly children” because their skin seems as fragile as a butterfly’s wings.


    Because the skin is so fragile, the wounds heal slowly. Some wounds may take months or years to heal. A severe wound may never heal.

    Blisters can also form on the tissue that lines the mouth, eyes, esophagus (tube that connects the throat to the stomach), intestines, urinary tract, or other areas of the body.

    The blistering ranges from mild to life-threatening.


    This is a common symptom of EB. Pain is often most intense for people who have severe EB.

    Causes of pain include the blisters and raw skin. An infection, bathing, bandage changing, and dehydrated skin can also cause pain.

    When blisters develop on tissue lining the mouth, eyes, and other areas inside the body, the pain can be unbearable.

    To cope with the pain, some people need pain medications every time their bandages are changed or just to get through the day.


    The blisters and wounds can lead to other health problems. The medical term for these other health problems is complications. This list of possible health problems is long and includes the following:

    • Anemia: This develops when too much blood oozes from the wounds. People with anemia have less oxygen in their body, which slows the healing of their skin even more.

    • Constipation: When we have a bowel movement, the tissue lining the rectum and anus stretches. If blisters develop in this tissue, bowel movements become extremely painful. To avoid this pain, a person may avoid having bowel movements. As a result, severe constipation develops. Diet changes may help eliminate this problem.

    • Dehydration: If EB causes large, open wounds, dehydration can occur.

    • Eye problems: Pink eye, swollen eyelids, and sores in the eyes along with pain and intense sensitivity to light are possible complications of EB. Diminished eyesight or blindness can occur.

    • Fingers (or toes) lose flexibility: If scars form as the skin heals, cycles of blisters and scarring on the hands or feet can cause the fingers or toes to stiffen.

      The scar tissue can become so thick that that fingers or toes fuse, becoming one. A bandaging technique can prevent this.

    • Hair loss: If scarring occurs repeatedly on the scalp, it can lead to hair loss.

    • Hardening and shortening of the muscles, tendons, and other tissues: As the skin repeatedly scars, this can cause the muscles, tendons, and other parts of the body to harden and shorten. Called contractures, these can make movement painful. Without treatment, contractures can lead to deformities.

    • Infection: Because the skin has open wounds, skin infections develop. Signs of a skin infection include redness, pain in the area, odor, and leaking of fluids like pus.

    • Itch: The skin can itch for many reasons, including inflamed skin, overheating, dryness, and healing wounds. Scratching the itchy skin can lead to intense pain.

    • Malnutrition: This is common when a person has severe EB. People often develop malnutrition because the body uses all the nutrition it gets to repair the skin. Children may fail to grow.

      Another cause of malnutrition is blisters in the mouth or esophagus. These blisters can make eating too painful. Medical care is often necessary to gain control over this problem.

    • Mobility problems: Blisters, pain, and scarring can make it painful to move. The less one moves, the more difficult it becomes to move.

    • Mouth sores, cavities, and problems opening the mouth: When blisters develop in the mouth, the pain can be excruciating. As the mouth heals, scars can limit how far one can open the mouth. The pain can prevent people with EB from brushing their teeth and seeing a dentist. Cavities and gum disease are common in people with EB.

    • Nail loss or deformed nails: EB can cause deformed nails. Some people with EB do not have nails.

    • Narrowing of the esophagus or another area: As scar tissue builds inside the body, this area can narrow. If the throat narrows, this can be life-threatening. Surgery can widen a narrowing throat.

    • Odor: The damaged skin can have an unpleasant odor, especially if a skin infection has developed.

    • Quality of life diminished: Trying to live with the blisters, ongoing medical care, and possible complications can take a toll on the parents and child. It can be difficult for a child to do well in school. Later in life, getting a job or driving a car may be challenging.

      This can create great hardship emotionally and financially.

    • Skin cancer: People who have severe forms of EB may have an increased risk of developing skin cancers, especially a type of skin cancer called squamous cell carcinoma. Skin cancer is more likely to occur when a person has recurrent or non-healing wounds.

    • Tiny, white bumps on the skin: Called milia (mill-ē-ah), these may appear when the blisters disappear.

    While the complications can vary greatly, what causes EB in children is the same. You’ll discover who’s most likely to get EB and what causes it at Epidermolysis bullosa (EB): Who gets and causes.

    Image 1: Getty Images

    Image 2: Used with permission of the American Academy of Dermatology National Library of Dermatologic Teaching Slides.

    El Hachem M, Zambruno G, et al. “Multicentre consensus recommendations for skin care in inherited epidermolysis bullosa.” Orphanet J Rare Dis. 2014 May 20;9:76.

    Jeon IK, On HR, et al. “Quality of life and economic burden in recessive dystrophic epidermolysis bullosa.” Ann Dermatol. 2016 Feb;28(1):6-14.

    Kudva P and Jain R. “Periodontal manifestation of epidermolysis bullosa: Looking through the lens.” J Indian Soc Periodontol. 2016 Jan-Feb;20(1):72-4.

    Pope E, Lara-Corrales I, et al. “A consensus approach to wound care in epidermolysis bullosa.” J Am Acad Dermatol. 2012 Nov;67(5):904-17.

    Pourreyron C, Cox G, et al. “Patients with recessive dystrophic epidermolysis bullosa develop squamous cell carcinoma regardless of type VII collagen expression.” J Invest Dermatol. 2007;127:2438-44.

    Watkins, J. “Diagnosis, treatment and management of epidermolysis bullosa.” Br J Nurs. 2016 Apr 28;25(8):428-31.

    Epidermolysis bullosa
  • Causes

    Epidermolysis bullosa (EB): Who gets and causes

    When a person has EB, the different layers of skin slip and slide against each other because the skin lacks the proteins needed to hold each layer in place.

    Who gets epidermolysis bullosa?

    EB is rare. It is estimated that between 25,000 and 50,000 people in the United States have EB1.

    Most types of EB begin in childhood. The fragile skin and blistering of EB are usually noticeable at birth or shortly thereafter. If a child has a mild case of EB, the blisters may begin when the child starts to crawl. Only one type of EB, epidermolysis bullosa acquisita (EBA), begins in adults.

    A child has a higher risk of developing EB if one or both parents carry genes for EB.

    As for race, EB develops in children of all races and ethnic groups.

    What causes epidermolysis bullosa?

    No type of EB is contagious. All but one type, epidermolysis bullosa acquisita (EBA), are caused by gene mutations. A mutation creates a slightly different version of a gene.

    When a child has EB, these slightly different genes mean the skin is missing the proteins needed to hold it together. Without these proteins, a gentle touch can cause the layers of skin to slide against each other, creating friction. It’s this friction that causes blisters to form.

    While a gentle touch can cause blisters to form, that’s not the case for everyone who has EB. The type of EB a person has determines how easily the skin blisters.

    When a child develops blisters in other areas, such as the mouth, throat, or rectum, it means that these areas are also missing the proteins that hold these layers together.

    The cause of EBA is a bit different. It’s thought that this type of EB is an autoimmune disease. When someone develops an autoimmune disease, the person’s immune system mistakes a part of the body as foreign and attacks it.

    When a person develops EBA, the body is attacking and destroying the proteins that hold the layers of skin together.

    Genetic counseling can identify EB genes

    Because EB can be such a painful and often disabling disease, people living with EB or who have EB in their family often want to spare their children from getting it. Genetic testing can tell you how likely it is that you will have a baby with EB. Testing can take several months, so it is best to do it before trying to have a child.

    It’s also possible to tell if a baby growing inside its mother has EB; however, this test can be risky for the baby.

    1Stanford University, Stanford Children’s Health. “FAQ about epidermolysis bullosa.” Last accessed July 2018.

    Getty Images

    Fine JD, “Epidermolysis bullosa.” In: Bolognia JL, et al. Dermatology. (second edition). Mosby Elsevier, Spain, 2008:457-66.

    Kudva P and Jain R. “Periodontal manifestation of epidermolysis bullosa: Looking through the lens.” J Indian Soc Periodontol. 2016 Jan-Feb;20(1):72-4.

    Epidermolysis bullosa
  • Treatment

    Epidermolysis bullosa (EB): Diagnosis, treatment, and outlook

    If your child has EB, you’ll often see signs of this disease, such as blistering or missing skin, when your child is born. The following explains how to get an accurate diagnosis, what treatment may involve, and the prognosis for a child born with EB.

    How is epidermolysis bullosa (EB) diagnosed?

    If a newborn has blisters or missing skin, it’s essential to find out why. While a child is still in the hospital, a dermatologist or pediatric dermatologist may be consulted. Other conditions aside from EB can cause blistering.

    To diagnose EB, a dermatologist will examine the child’s skin closely, looking at the blisters and other signs. Your dermatologist will also examine other parts of the child’s body to see if they are affected.

    During this evaluation, you should be prepared to answer questions, such as “Does anyone in your family have EB or blistering skin?”

    After the exam and questioning, your dermatologist will remove a small part of a blister. This sample is called a “skin biopsy.” It will be sent to a lab that has the equipment required to diagnose EB. This testing is required to find out whether a child has EB. A specialized test may be able to determine the type of EB the child has.

    Genetic testing may be also be recommended. This can tell you which genes are causing the EB. For this genetic test, blood needs to be drawn.

    How is EB treated?

    There is no cure for EB, so EB may require lifelong care. When possible, a person with EB is usually referred to an EB treatment center. There are located across the United States.

    Treatment for EB consists of:

    • Wound care
    • Pain management
    • Preventing new injuries
    • Watching for and treating complications

    At an EB treatment center, you’ll find specialists from different areas of medicine who have experience with EB. Specialists include pediatricians, dermatologists, pediatric dermatologists, nurses, dieticians, and psychologists. They can help with the extensive care that a patient with EB often needs.

    If you cannot go to an EB treatment center, you should see a doctor who has experience with EB, such as a pediatric dermatologist or dermatologist. In this case, your dermatologist may be the primary doctor you see about EB. The dermatologist will refer you to other specialists as needed.

    The amount of care a person with EB needs varies. Parents who have a child with mild EB may be able to care for the child without much help once they learn how to treat the wounds and prevent new injuries. More severe EB requires professional medical help.

    When caring for someone who has EB, treatment usually involves:

    • Wound care: Caring for wounds can take an hour or longer each day. Most patients must wear bandages that must be changed every day or every other day.

      If EB is severe, a patient may need a nurse’s help with wound care. A nurse may go to the home of a patient with EB to help with wound care.

    • Pain management: People with EB are often in pain. So many things can cause pain, including the blisters and raw skin, an infection, swallowing, eating, urinating, having a bowel movement, or just moving about.

      Medication is often needed to relieve the pain. Antidepressants, medicine used to treat epilepsy, and acetaminophen can be helpful. If the pain is severe, medicine like fentanyl, morphine, or ketamine can be prescribed.

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

    • Preventing new injuries: Using padded bandages and wearing loose-fitting clothes can help prevent new wounds.

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

    • Watching for and treating complications: The blisters and wounds can lead to other health problems, which your doctor may call complications. Treatment for the complications may involve:

      • Anemia: Good wound care and preventing (and treating) an infection can reduce blood loss, which leads to anemia. When anemia develops, iron supplements, giving iron intravenously, or a blood transfusion can treat it.

      • Cavities and other problems in the mouth: Gum disease and cavities are common in people who have EB. Patients may need to see a dentist who has experience treating EB every 3 months.

      • Constipation: EB can make a bowel movement excruciatingly painful. A dietitian may be able to prevent constipation and other problems like diarrhea by changing the patient’s diet.

      • 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 together.

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

      • Dry skin: Applying a moisturizer every day can help reduce dry skin, which can reduce blistering, pain, and itching. If a nurse helps with wound care, the nurse can apply the moisturizer.

      • Excess sweating: Common in patients with a type of EB known as epidermolysis bullosa simplex (EBS), excessive sweating should be treated. Treating it can reduce blistering and itching.

      • Eye problems: Sores on the eye, blistering eyelids, and other eye problems can occur. The sooner these are treated, the better.

      • 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, infections occur. Treatment often involves changing bandages daily and 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 because eating becomes too painful. A dietitian may be able to recommend foods that a child can eat; however, sometimes the child needs a feeding tube.

      • Non-healing wounds: A special wound covering can help. Some chronic wounds, however, will not fully close. No one knows why this happens.

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

      • 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: Usually beginning in a patient’s 20s or 30s, skin cancer can develop in areas with long-standing wounds and scars. The goal of treatment is to remove all of the cancer. Surgery is often used; however, other types of treatment for skin cancer may be necessary.

    Researchers are trying to improve treatment

    While today’s treatment relies on wound care, pain management, and treating complications, researchers are looking for ways to improve this — and possibly cure EB. They are making great progress in this area.

    What is the outlook for someone who has EB?

    This varies with the severity of the EB.

    If a child has mild EB, the EB may become less severe with age. As an adult, life may be somewhat normal.

    At the other end of the spectrum, EB can require hours of care each day for life. A nurse’s help may be necessary to bathe and change bandages. The patient may frequently see medical doctors from different specialties including dermatology, ophthalmology, gastroenterology, and hematology.

    Severe EB can be life-threatening. Widespread blistering, infections, and loss of body fluid can cause serious problems. Some children die of EB.

    Some people who have severe EB die in their 20s or 30s of a type of skin cancer called squamous cell carcinoma. When this skin cancer develops in the wounds, it can be very aggressive. This makes it hard to successfully treat.

    While these generalizations apply, it’s important to remember that each person who has EB is different. You cannot always predict what will happen.

    Images used with permission of the American Academy of Dermatology National Library of Dermatologic Teaching Slides.

    Castillo RO, Davies YK, et al. “Management of esophageal strictures in children with recessive dystrophic epidermolysis bullosa.” J Pediatr Gastroenterol Nutr. 2002 May;34(5):535-41.

    El Hachem M, Zambruno G, et al. “Multicentre consensus recommendations for skin care in inherited epidermolysis bullosa.” Orphanet J Rare Dis. 2014 May 20;9:76.

    Fine, JD. “Epidermolysis bullosa.” In: Bolognia JL, et al. Dermatology. (second edition). Mosby Elsevier, Spain, 2008:457-66.

    Fine JD, Bruckner-Tuderman L, et al. “Inherited epidermolysis bullosa: Updated recommendations on diagnosis and classification.” J Am Acad Dermatol. 2014 Jun;70(6):1103-26.

    Jeon IK, On HR, et al. “Quality of life and economic burden in recessive dystrophic epidermolysis bullosa.” Ann Dermatol. 2016 Feb;28(1):6-14.

    Leal SC, Lia EN, et al. “Higher dental caries prevalence and its association with dietary habits and physical limitation in epidermolysis bullosa patients: A case control study.” J Contemp Dent Pract. 2016 Mar 1;17(3):211-6.

    Oliveira TM, Sakai VT, et al. “Clinical management for epidermolysis bullosa dystrophica. J Appl Oral Sci. 2008 Jan-Feb;16(1):81-5.

    Pope E, Lara-Corrales I, et al. “A consensus approach to wound care in epidermolysis bullosa.” J Am Acad Dermatol. 2012 Nov;67(5):904-17.

    Scheidt L, Sanabe ME, et al. “Oral Manifestations and Dental Management of Epidermolysis Bullosa Simplex.” Int J Clin Pediatr Dent 2015;8(3):239-241.

    Watkins, J. “Diagnosis, treatment and management of epidermolysis bullosa.” Br J Nurs. 2016 Apr 28;25(8):428-31

    Epidermolysis bullosa
  • Tips

    Epidermolysis bullosa (EB): Tips dermatologists give parents

    Some children, such as this boy who has severe EB, look forward to spending a week at Camp Discovery all year long.

    Dermatologists are committed to improving the quality of life for children who have EB and their parents. One way they do this is by giving parents information to help them care for their child.

    These tips include the following:

    1. Try to reduce blisters from diapers and clothes with these tips:

    • Use disposable diapers with Velcro fasteners instead of tapes, which could stick to the child’s skin.

    • Line each disposable diaper with a product called a silicone contact sheet if the diaper area is fragile. This product won’t stick to wounds. You can find it at medical supply companies.

    • Dress your baby in a one-piece lightweight garment that fastens in the front. This can make it easier to dress and undress your child.

    • Turn clothing inside-out so that the seams show. Sometimes, when seams rub against the skin, blisters form.

    • Use special therapeutic clothing that has been tested in clinical trials and shown to be less irritating than other clothing.

    1. Prevent medical gloves from injuring your child’s skin. The latex or vinyl may rub your child’s skin, which can cause blisters. To help prevent this, dermatologists recommend:

    • If you can, try to stop wearing gloves during wound care: Instead of wearing gloves, wash your hands frequently while caring for your child’s skin.

    • When gloves are a necessity: Apply an ointment, such as pure petroleum jelly, to the gloves. This helps reduce friction.

    1. Ask your child’s dermatologist to recommend skin care products if you experience a problem with one. Lotions, creams, and ointments help keep your child more comfortable, and your child’s dermatologist can be an excellent source for product recommendations if:

    • A skin care product becomes less effective after a period of time

    • Your child needs an anti-itch product

    • A wound becomes extremely stubborn and is not responding to the usual ointment

    1. Report complications as soon as they arise. Blisters can develop just about anywhere, and sometimes scars appear when the skin heals. Both can lead to unexpected complications.

      With early intervention, many complications can be lessened or prevented. Here are a few examples to illustrate why it’s so important to report complications as they occur:

    • Scarring on the hands or feet can cause the fingers or toes to join together, but learning a new bandage wrapping technique can prevent or delay this.

    • Blisters in the rectum can be so painful that a child holds back on bowel movements. Changing what you feed your child can ease the pain, allowing your child to have bowel movements.

    • Dryness in the eyes can lead to a scratched cornea, but using eye drops or eye ointments may prevent this and other eye problems.

    1. Encourage play and movement. It’s understandably hard to encourage your child to play and move about when the skin is so fragile, but children with EB should move as much as they can. It is better for a child to get a blister now and again than to be too afraid to move.

      Moving around helps to:

    • Prevent constipation.

    • Reduce the shortening and thickening of muscles, tendons, and other tissues, which can make it difficult if not impossible to move the affected area of the body.

    • Stop muscle wasting, as muscles can shrink and weaken without movement.

    1. Make appointments for full-body skin exams. Regardless of the type of EB your child has, dermatologists recommend full-body skin exams beginning at age 10. Problems can develop when wounds:

    • Occur again and again
    • Cause pain
    • Stay the same for longer than 1 month

    One such problem is an increased risk of developing skin cancer. For this reason, dermatologists recommend that a child who has EB have full-body skin exams as often as shown below.

    Full-body skin exam
    10 to 16 years old
    Every 3 to 6 months
    16 years and older
    Every 3 months

    1. Keep learning about EB. The more you know, the better you can care for your child. A recommended resource for learning more about EB is EBnurse.org. On this site, you can access the latest information, ask an EB nurse questions, learn from other EB families, and pick up tips for caring for your child.

    2. Try to have your child evaluated at one of the EB clinics in the United States at some point. Being seen by a team of specialists at an EB clinic helps to ensure that your child gets the required care and that you are aware of available resources.

      During an evaluation, your child may be seen by a dermatologist who has expertise in EB along with specialists in gastroenterology, nutrition, dentistry, hematology, hand or plastic surgery, orthopedics, ophthalmology, social services, pain management, and physical therapy.

      To date, there are 4 EB clinics in the United States that offer this multidisciplinary approach to care:

    • Children’s Hospital Colorado
    • Cincinnati Children’s Hospital
    • Lucille Packard Children’s Hospital (Stanford University in California)
    • Phoenix Children’s Hospital

    1. Consider sending your child to Camp Discovery for one week. It can be frightening to think about being away from your child for a week, especially if your child needs daily medical care. Camp Discovery is equipped to handle these needs while giving your child an opportunity to experience what it’s like to go to summer camp. Many true friendships begin at Camp Discovery, and many kids look forward to camp all year long.

      “I tried archery, even though my hands are bandaged. I rode a horse – which was awesome – and I even got to go to a dance.”

      – Henry, 3rd grader with EB

      To make Camp Discovery accessible to children living with a chronic skin condition, the American Academy of Dermatology:

    • Staffs each camp with medical professionals, including dermatologists, pediatricians, physician assistants, and nurses.

    • Sets up each location to handle daily medical care regimens and medical emergencies.

    • Charges nothing to attend, even the costs of transportation are covered.

    You can find out more at Camp Discovery.

    American Academy of Dermatology

    American Academy of Dermatology, “Changing children’s lives one summer at a time for 20 years.” News release issued June 20, 2013.

    Badger KS, O’Haver J, et al. “Recommendations for a comprehensive management plan for the child diagnosed with epidermolysis bullosa.” J Dermatol Nurses’ Assn. 2013:5(2):72-8.

    Harris, A, Jain S, et al. “Poster 1594: The Epidermolysis Bullosa Disease Activity and Scarring Index (EBDASI) mobile phone application.” J Am Acad Dermatol. 2015:72(5):AB122. Commercial support: None identified.

    Krämer SM, Serrano MC, et al. “Oral health care for patients with epidermolysis bullosa - Best clinical practice guidelines.” Int J Paediatr Dent. 2012:22 (Suppl. 1): 1–35.

    Watkins, J. “Diagnosis, treatment and management of epidermolysis bullosa.” Br J Nurs. 2016 Apr 28;25(8):428-31.

    Epidermolysis bullosa