Epidermolysis bullosa

Injury	Disease	Nutrition	Poison

Symptoms	Surgery	Test	Special Topic

Epidermolysis bullosa, dominant dystrophic

Epidermolysis bullosa, dystrophic

Epidermolysis bullosa

Alternative Names:

Junctional epidermolysis bullosa; Dystrophic epidermolysis bullosa; Hemidesmosomal epidermolysis bullosa; Weber-Cockayne syndrome
Treatment:

The goal of treatment is to prevent the formation of blisters and subsequent complications. The intensity of care depends upon the severity of the disease. Recommendations often include measures to avoid skin trauma and to avoid high environmental temperatures.

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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 application of topical antibiotics 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, however. If candida is present 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 followed by a dentist with experience treating those with epidermolysis bullosa.

Proper nutrition is also important. When skin injury is extensive, increased calorie and protein intake may be necessary to help recovery. Work closely with a nutritionist. When blisters or complications are present in the mouth or esophagus, hard or brittle foods (such as pretzles, nuts, and chips) should be avoided. Eating soft foods can help prevent worsening of these lesions.

Working with a physical therapist can help maintain the range of motion of involved joint areas 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 as well. Such surgeries include dilation of the esophagus if a stricture (narrowing) is present, repair of hand deformities, and removal of squamous cell carcinoma lesions if they 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, particularly the latter drug category. Studies using interferon are also underway.

Expectations (prognosis):