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Epidermolysis Bullosa - Definition, Causes, Symptoms and Treatment


Definition

Epidermolysis bullosa is also known as junctional epidermolysis bullosa, Dystrophic epidermolysis bullosa, Hemidesmosomal epidermolysis bullosa, Weber-Cockayne syndrome etc. Epidermolysis bullosa is a group of inherited bullous disorders characterized by blister formation in response to mechanical trauma. The skin is so fragile in people with EB that even minor rubbing may cause blistering. At times, the person with EB may not be aware of rubbing or injuring the skin even though blisters develop. In severe EB, 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. EB is an inherited disease that affects less than 10,000 children and adults in the United States.

Epidermolysis Bullosa is of four types

  • Epidermolysis bullosa simplex
  • Junctional epidermolysis bullosa
  • Dystrophic epidermolysis bullosa
  • Hemidesmosomal epidermolysis bullosa

Causes

  1. Heredity: EB have inherited the condition through faulty genes they receive from one or both parents. Genes are located in the body's cells and determine inherited traits passed from parent to child.
  2. The disease results when skin layers separate after minor trauma.
  3. Defects of several proteins within the skin are at fault.

Symptoms

  1. Blistering in or around the mouth and throat, causing feeding difficulty or swallowing difficulty.
  2. A hoarse cry, cough, or other respiratory difficulties
  3. In the most serious forms, large, ulcerated blisters on the face, trunk, and legs can be life-threatening due to complicated infections and loss of body fluid that leads to severe dehydration.
  4. The teeth of children with EB are often bad as a result of the disrupted enamel formation.
  5. The child sometimes displays voiding problems when blisters or blood clots obstruct the urinary tract. This may lead to urinary retention and finally impaired renal function because of which dialysis is required.

Treatment

  1. Wound healing: This process is impaired by multiple factors including foreign bodies, bacteria, nutritional deficiencies, tissue anoxia, and aging. Exogenous agents contributing to impairment of wound healing include glucocorticoids and penicillamine.
  2. Gene therapy: The goal is to deliver genes targeted to restore normal protein production so that this disease is control.
  3. One medical approach is to use phenytoin and oral steroid elixirs to reduce the symptoms of dysphagia. In addition, if oral candidiasis is present, an anticandidal medication is helpful.
  4. Obtaining and maintaining a good nutritional status.
  5. Use foam padding or sheepskins to help reduce friction on furniture such as beds, chairs and infant car seats.
  6. Try to avoid using nappies in infants with severe EB, instead place child on a clean pad.
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