Treatment
The goal of treatment is to prevent the formation of blisters and subsequent complications. How much treatment is needed depends on how severe the disease is. Recommendations often include measures to avoid skin trauma (damage) and to avoid hot environments.
To prevent infection, excellent skin care is required, especially if any blistered areas become crusted or denuded (exposed or raw). Follow the instructions of your health care provider closely. Recommendations might include regular whirlpool therapy and applying antibiotic ointments to these wound-like areas. Your health care provider will let you know if a bandage or dressing is required, and what type.
If there are swallowing difficulties, use of oral steroids for short periods of time may be prescribed. Long term use of steroids for epidermolysis bullosa is generally not recommended. If there is candida in the mouth or esophagus, medication for that secondary infection would be prescribed as well.
Good dental hygiene is very important, including regular dental visits. It is best to be under the ongoing care of a dentist who has experience treating people with epidermolysis bullosa.
Proper nutrition is also important. When there is a lot of skin injury, you may need extra calories and protein to help recovery. Work closely with a nutritionist. When there are blisters or complications in the mouth or esophagus, avoid eating hard or brittle foods such as pretzels, nuts, and chips. Eating soft foods can help prevent making the sores worse.
Working with a physical therapist can help keep the full range of motion in the joints and minimize contractures.
Skin grafting for denuded or ulcerated areas of the skin may be necessary. Other surgical procedures for complications of epidermolysis bullosa might be recommended. Such surgeries include dilation of the esophagus if there is a stricture (narrowing), repair of hand deformities, and removal of of any squamous cell carcinoma lesions that develop.
Other treatments under investigation for epidermolysis bullosa include protein and gene therapy.
Epidermolysis bullosa acquisita may be treated with oral steroids and medication that suppresses the immune system. These may, however, increase the risk of secondary infection. Studies using interferon are also underway.
Prognosis (Expectations)
What happens depends on how severe the illness is. Mild forms of epidermolysis bullosa improve with age. Scarring from several types of epidermolysis bullosa can restrict mobility and impair daily activities. Lethal forms of epidermolysis bullosa have a very high mortality rate. (See Complications section.)
Complications
- Anemia
- Death — the death rate is as high as 87% in the first year of life for infants with the lethal form of junctional epidermolysis bullosa. On the other hand, dominantly inherited simplex and dystrophic epidermolysis bullosa, and mild forms of junctional epidermolysis bullosa may not reduce life expectancy at all.
- Esophageal stricture
- Eye disorders, even blindness
- Infection, including sepsis
- Loss of function of the hands and feet
- Muscular dystrophy
- Periodontal disease
- Severe malnutrition caused by feeding difficulty, leading to failure to thrive
- Squamous cell skin cancer — if someone with epidermolysis bullosa survives childhood, metastatic squamous cell cancer of the skin is the most common cause of death. This skin cancer occurs in those with recessively inherited dystrophic epidermolysis bullosa. Those with epidermolysis bullosa often develop this skin cancer between age 15 and 35. In addition, the skin cancer can occur anywhere on the skin. In other words, unlike in the general public, it is not more likely to happen in sun-exposed areas.
Calling Your Health Care Provider
If your infant exhibits any blistering shortly after birth call your health care provider. If you have a family history of epidermolysis bullosa and plan to have children, genetic counseling may be appropriate.
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Epidermolysis bullosa : Overview, Causes, & Risk Factors
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Epidermolysis bullosa : Symptoms & Signs, Diagnosis & Tests
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Epidermolysis bullosa : Treatment
Review Date : 10/31/2008
Reviewed By : Kevin Berman, MD, PhD, Atlanta Center for Dermatologic Disease, Atlanta, GA. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.
The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only — they do not constitute endorsements of those other sites. © 1997- 2010 A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.
