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Definition of «Epidermolysis bullosa»

Epidermolysis bullosa: One in a group of blistering skin conditions. The skin is so fragile in people with epidermolysis bullosa that even minor rubbing may cause blistering. At times, the person may not be aware of rubbing or injuring the skin even though blisters develop. In severe epidermolysis bullosa, blisters are not confined to the outer skin. They may develop inside the body, in such places as the linings of the mouth, esophagus, stomach, intestines, upper airway, bladder, and the genitals. Most forms of epidermolysis bullosa are evident at birth. This disorder can be both disabling and disfiguring, and some forms may lead to early death. The disease results when skin layers separate after minor trauma. Defects of several proteins within the skin are at fault.

To pick up a baby with severe epidermolysis bullosa, place the child on a soft material and support the under the buttocks (bottom) and behind the neck. A baby with epidermolysis bullosa should never be picked up under the arms. To protect the skin from injury, avoid overheating by keeping rooms at an even temperature, apply lubricants to the skin to reduce friction and keep the skin moist, use simple, soft clothing that requires minimal handling when dressing a child, use sheepskin on car seats and other hard surfaces, and put mittens on at bedtime to help prevent scratching. When blisters appear, the objectives of care are to reduce pain or discomfort, prevent excessive loss of body fluid, promote healing, and prevent infection. If infection appears, it should be promptly treated.

See also: Blister; Epidermolysis bullosa simplex; Junctional epidermolysis bullosa; Dystrophic epidermolysis bullosa; Epidermolysis bullosa acquisita.

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