Epidermolysis bullosa (EB) is a rare disorder that causes the skin — and sometimes surfaces inside the body like the intestines — to break and blister easily. This leads to pain, discomfort, and sometimes fatal complications. Symptoms of EB generally start at a young age.
There is currently no cure for EB and treatments focus on preventing blisters or managing the wounds caused by the condition. There are different types of EB that are associated with blisters forming in different locations on the body and that vary in severity. Different treatments are used depending on the type of epidermolysis bullosa.
The first step in epidermolysis bullosa treatment is reducing the number of blisters that form. Patients or their parents can make changes to their lifestyle, such as:
- Keep the temperature cool;
- Choose soft, loose clothing that is easy to take on and off;
- Avoid walking long distances;
- Prevent scratching by trimming fingernails and wearing mittens to bed;
- Avoid hard or rough surfaces by covering them with a soft material, such as sheepskin.
Applying lubricants or moisturizers may help keep the skin moist and reduce friction. However, this should be discussed with a specialist beforehand.
Physical contact can cause blisters, but physical contact should not be avoided, nor should the patient stop taking part in physical activities. Specialists can advise parents and caregivers on ways to safely hold and play with children who have EB and which activities are least likely to result in blistering.
When blisters occur, the correct care can speed healing, prevent infections, and reduce scarring. Medical professionals can provide training on how to safely pierce and drain blisters, which can prevent them from getting too large before they break.
It is essential to keep wounds clean to prevent infections — for example, by soaking them in a mild salt solution. Wounds can be covered with sterile bandages to help prevent infections, and bandages can help create a moist environment which can promote healing. Some bandages can cause irritation or additional skin damage. There are special non-sticking bandages that may help (such as Mepilex or PolyMem).
There are no EB-specific medications. Depending on the severity of the condition, doctors may recommend over-the-counter pain medication or may prescribe stronger painkillers, such as morphine, if needed. If the patient is experiencing chronic pain, then medicines such as amitriptyline, gabapentin or pregabalin may be prescribed.
If a wound becomes infected, it may be treated with antibiotics. These can be applied as a topical lotion, a soaking solution, or taken as an oral tablet.
In some cases, surgery may be an option to improve general quality of life.
In cases where repeated blistering has occurred, scar tissue can cause deformities such as fused fingers and toes that result in reduced mobility. Surgery may be required to separate them.
Weight loss can be an issue in some patients with EB. Scarring caused by blisters can result in a narrowing of the esophagus (the tube connecting the mouth and stomach), making it difficult for food to reach the stomach. Surgery to widen the esophagus can improve this. Insertion of a feeding tube can help improve poor weight gain and nutrition, as food can be delivered directly to the stomach, and this can be maintained along with eating through the mouth.
Note: Epidermolysis Bullosa News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website.