BACK TOTOP Browse A-ZSearchBrowse A-ZABCDEFGHIJKLMNOPQRSTUVWXYZ0-9 E-mail FormEmail ResultsName:Email address:Recipients Name:Recipients address:Message: Print-FriendlyBookmarksbookmarks-menuEpidermolysis bullosaEB; Junctional epidermolysis bullosa; Dystrophic epidermolysis bullosa; Hemidesmosomal epidermolysis bullosa; Weber-Cockayne syndrome; Epidermolysis bullosa simplexEpidermolysis bullosa (EB) is a group of disorders in which skin blisters form after a minor injury. It is passed down in families. Causes There are 3 main types of EB. They are:Dystrophic epidermolysis bullosa Epidermolysis bullosa simplex Junctional epidermolysis bullosaEB can vary from minor to fatal. The minor forms causes blistering of the skin. The fatal forms affect other organs. Most types of this condition start at birth or soon after. It can be hard to identify the exact type of EB a person has, although specific genetic markers are now available for most.Family history is a risk factor. The risk is higher if a parent has this condition.Another rare type of EB is called epidermolysis bullosa acquisita. This form develops after birth. It is an autoimmune disorder, which means the body attacks itself. Symptoms Depending on the form of EB, symptoms can include:Alopecia (hair loss) Blisters around the eyes and nose BlistersA vesicle is a small fluid-filled blister on the skin.ImageRead Article Now Book Mark Article Blisters in or around the mouth and throat, causing feeding problems or swallowing difficulty Blisters on the skin as a result of minor injury or temperature change, especially of the feet Blistering that is present at birth Dental problems, such as tooth decay Hoarse cry, cough, or other breathing problems Tiny white bumps on previously injured skin Nail loss or deformed nails Mitten-like hands or feet Thickening of skin on hands and feet Muscle, heart, brain, gastrointestinal, bone, or kidney issues Exams and Tests Your health care provider will look at your skin to diagnose EB.Tests that are used to confirm the diagnosis include:Genetic testing Skin biopsy Skin biopsyA skin lesion biopsy is when a small amount of skin is removed so it can be examined. The skin is tested to look for skin conditions or diseases. A...ImageRead Article Now Book Mark Article Special tests of skin samples under a microscope Skin tests may be used to identify the form of EB.Other tests that may be done include:Blood test for anemia Culture to check for bacterial infection if wounds are healing poorly Imaging studies to look at other organsGrowth rate will be checked often for a baby who has or may have EB. Treatment The goal of treatment is to prevent blisters from forming and avoiding complications. Other treatment will depend on how bad the condition is.HOME CAREFollow these guidelines at home:Take good care of your skin to prevent infections. Follow your provider's advice if blistered areas become crusted or raw. You might need regular whirlpool therapy and to apply antibiotic ointments to wound-like areas. Your provider will let you know if you need a bandage or dressing, and if so, what type to use. You may need to use oral steroid medicines for short periods of time if you have swallowing problems. You may also need to take medicine if you get a candida (yeast) infection in the mouth or throat. Take good care of your oral health and get regular dental check-ups. It is best to see a dentist who has experience treating people with EB. Eat a healthy diet. When you have a lot of skin injury, you may need extra calories and protein to help your skin heal. Choose soft foods and avoid nuts, chips, and other crunchy foods if you have sores in your mouth. A nutritionist can help you with your diet. Do exercises a physical therapist shows you to help keep your joints and muscles mobile.SURGERYSurgery to treat this condition may include:Skin grafting in places where sores are deep Skin graftingA skin graft is a patch of skin that is removed by surgery from one area of the body and transplanted, or attached, to another area.ImageRead Article Now Book Mark Article Dilation (widening) of the esophagus if there is a narrowing Repair of hand deformities Removal of any squamous cell carcinoma (a type of skin cancer) that developsOTHER TREATMENTSOther treatments for this condition may include:Medicines that suppress the immune system may be used for the autoimmune form of this condition. Protein and gene therapy and the use of the drug interferon are being studied. Outlook (Prognosis) The outlook depends on the severity of the illness. Infection of the blistered areas is common.Mild forms of EB improve with age. Very serious forms of EB have a very high death rate.In the severe forms, scarring after blisters form may cause:Contracture deformities (for example, at the fingers, elbows, and knees) and other deformities Contracture deformitiesA contracture develops when the normally stretchy (elastic) tissues are replaced by nonstretchy (inelastic) fiber-like tissue. This tissue makes it ...ImageRead Article Now Book Mark Article Swallowing problems if the mouth and esophagus are affected Fused fingers and toes Limited mobility from scarring Possible Complications These complications may occur:Anemia AnemiaAnemia is a condition in which the body does not have enough healthy red blood cells. Red blood cells provide oxygen to body tissues. Different type...ImageRead Article Now Book Mark Article Reduced life span for severe forms of the condition Esophageal narrowing Esophageal narrowingBenign esophageal stricture is a narrowing of the esophagus (the tube from the mouth to the stomach). It causes swallowing difficulties. Benign mean...ImageRead Article Now Book Mark Article Eye problems, including blindness Infection, including sepsis (infection in the blood or tissues) SepsisSepsis is an illness in which the body has a severe, inflammatory response to bacteria or other germs.ImageRead Article Now Book Mark Article Loss of function in the hands and feet Muscular dystrophy Muscular dystrophyMuscular dystrophy is a group of inherited disorders that cause muscle weakness and loss of muscle tissue, which get worse over time.ImageRead Article Now Book Mark Article Periodontal disease Periodontal diseasePeriodontitis is inflammation and infection of the ligaments and bones that support the teeth.ImageRead Article Now Book Mark Article Cardiac problems Kidney failure Severe malnutrition caused by feeding difficulty, leading to failure to thrive Failure to thriveFailure to thrive refers to children whose current weight or rate of weight gain is much lower than that of other children of similar age and sex....Read Article Now Book Mark Article Squamous cell skin cancerSquamous cell skin cancerSquamous cell cancer is the second most common type of cancer in the United States. Other common types of skin cancer are:Basal cell cancerMelanoma...ImageRead Article Now Book Mark Article When to Contact a Medical Professional If your infant has any blistering shortly after birth, call your provider. If you have a family history of EB and plan to have children, you may want to have genetic counseling. Prevention Genetic counseling is recommended for prospective parents who have a family history of any form of epidermolysis bullosa.During pregnancy, a test called chorionic villus sampling may be used to test the baby. For couples at high risk of having a child with EB, the test can be done as early as week 8 to 10 of pregnancy. Talk to your provider.Chorionic villus samplingChorionic villus sampling (CVS) is a test some pregnant women have to screen their baby for genetic problems.ImageRead Article Now Book Mark Article To prevent skin damage and blistering, wear padding around injury-prone areas such as the elbows, knees, ankles, and buttocks. Avoid contact sports.If you have EB acquisita and are on steroids for longer than 1 month, you may need calcium and vitamin D supplements. These supplements may help prevent osteoporosis (thinning bones).OsteoporosisOsteoporosis is a disease in which bones become fragile and more likely to break (fracture).ImageRead Article Now Book Mark Article Open ReferencesReferencesDenyer J, Pillay E, Clapham J. Best Practice Guidelines for Skin and Wound Care in Epidermolysis Bullosa: An International Consensus. London, UK: Wounds International; 2017. www.woundsinternational.com/resources/details/best-practice-guidelines-skin-and-wound-care-in-epidermolysis-bullosa.Dinulos JGH. Vesicular and bullous diseases. In: Dinulos JGH, ed. Habif's Clinical Dermatology. 7th ed. Philadelphia, PA: Elsevier; 2021:chap 16.Fine J-D, Mellerio JE. Epidermolysis bullosa. In: Bolognia JL, Schaffer JV, Cerroni L, eds. Dermatology. 4th ed. Philadelphia, PA: Elsevier Limited; 2018:chap 32.AllVideoImagesTogEpidermolysis bullosa, dominant dystrophic - illustration This picture shows skin lesions (epidermolysis bullosa) over the joints on the hands and feet (interphalangeal joints). Dystrophic epidermolysis bullosa is an inherited condition that causes red, blisters (bullae) that break open, ooze, form scabs (crusts), and scar.Epidermolysis bullosa, dominant dystrophicillustrationEpidermolysis bullosa, dystrophic - illustration This picture shows skin lesions (epidermolysis bullosa) over the joints on the hands and feet (interphalangeal joints). Dystrophic epidermolysis bullosa is an inherited condition that causes red, blisters (bullae) that break open, ooze, form scabs (crusts), and scar.Epidermolysis bullosa, dystrophicillustrationEpidermolysis bullosa, dominant dystrophic - illustration This picture shows skin lesions (epidermolysis bullosa) over the joints on the hands and feet (interphalangeal joints). Dystrophic epidermolysis bullosa is an inherited condition that causes red, blisters (bullae) that break open, ooze, form scabs (crusts), and scar.Epidermolysis bullosa, dominant dystrophicillustrationEpidermolysis bullosa, dystrophic - illustration This picture shows skin lesions (epidermolysis bullosa) over the joints on the hands and feet (interphalangeal joints). Dystrophic epidermolysis bullosa is an inherited condition that causes red, blisters (bullae) that break open, ooze, form scabs (crusts), and scar.Epidermolysis bullosa, dystrophicillustrationRelated Information Autoimmune disorders(Condition)Autosomal dominant(Special Topic)Autosomal recessive(Special Topic)Vesicles(Symptoms)Chafing(Special Topic)Contracture deformity(Symptoms)Secondary infections(Special Topic)Sepsis(Condition)Esophageal stricture – benign(Condition)Periodontitis(Condition)Anemia(In-Depth) Review Date: 11/4/2020 Reviewed By: Elika Hoss, MD, Senior Associate Consultant, Mayo Clinic, Scottsdale, AZ. 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