Treatment of epidermolysis bullosa aims mainly at preventing complications and easing discomfort from blistering.
Blisters may be large and, once broken, susceptible to infection and fluid loss. Your doctor may recommend the following tips for treating blisters and raw skin:
- Puncture blisters with a sterile needle to prevent the blister from spreading further. Leaving the roof of the blister intact allows for drainage of the blister while protecting the underlying skin.
- Apply antibiotic ointment, petroleum jelly or other moisturizing substance before applying a special nonsticking bandage.
- Soak wounds with a disinfectant solution. For wounds that don't heal, infection with bacteria such as pseudomonas may be playing a role. Soaks with diluted vinegar solution are sometimes used as a disinfectant, starting with a low enough concentration that the solution doesn't sting but is still helpful to remove germs.
Ideally, deformities and fusion of the hands and feet can be prevented with daily protective wrapping. However, repeated blistering and scarring can cause deformities, such as fusing of the fingers or toes or abnormal bends in the joints (contractures). Your doctor may recommend surgery to correct these deformities, particularly if they interfere with normal motion.
Blistering and scarring of the esophagus may lead to esophageal narrowing, making eating difficult. Surgery to widen (dilate) the esophagus may be needed. Using light sedation, the surgeon positions a small balloon in the esophagus and inflates it to dilate the area.
To improve nutrition and help with weight gain, a tube (gastrostomy tube) may be implanted to deliver food directly to the stomach. Feedings through the tube may be delivered overnight using a pump. Eating through the mouth is continued if possible so that the child will be able to eat with others for normal socializing.
Working with a physical therapist can help ease the limitations on motion caused by scarring and shortening of the skin (contracture). Swimming may be helpful for many people.
Intensive studies are under way to find better ways to treat and relieve the symptoms of epidermolysis bullosa, including gene replacement, bone marrow transplantation and recombinant protein therapies.