Epidermolysis Bullosa

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Epidermolysis Bullosa (EB) is a rare genetic disease that causes the skin to blister and peel. These blisters form at the slightest touch and may appear in response to minor injury, heat or friction from rubbing, scratching, or adhesive tape. In severe cases, the blisters may occur inside the body, such as the lining of the mouth and intestines.

Symptoms

Very fragile skin with tendency to develop blisters after mild trauma.

Other signs and symptoms of EB may include:

  • fluid-filled blisters on the skin, especially on the hands and feet due to friction
  • deformity or loss of fingernails and toenails (nail dystrophy)
  • internal blistering, including on the vocal cords, oesophagus and upper airway
  • skin thickening on the palms and the soles of the feet
  • scalp blistering, scarring and hair loss (scarring alopecia)
  • thin-appearing skin (atrophic scarring)
  • tiny white skin bumps or pimples (milia)
  • dental problems, such as tooth decay from poorly-formed enamel
  • difficulty swallowing (dysphagia)
  • nutrition problems or poor growth can also occur, mainly if the blisters are in the oesophagus or stomach – blisters in these areas can make it painful to eat

Causes

EB is a genetic disease and in most cases the condition is inherited. It usually shows up during infancy or early childhood.

Treatments

Although there is no cure for EB, patients can follow treatment plans by their doctor to help prevent blisters from forming.

The main treatment for EB is good wound care, to prevent infection while the skin heals.

To prevent blisters:

  • Wear soft clothing made from natural fibres to avoid rubbing on and irritating the skin.
  • Try not to get too hot.
  • Wear soft mittens at night to prevent scratching.
  • Use sheepskin or foam padding on surfaces that may cause friction, e.g., chairs, beds, infant car seats.

EB patients must see their doctor if their blister(s) become infected. If an infection has occurred, a doctor may:

  • recommend an antibiotic cream
  • recommend antibiotics
  • recommend a special covering for blisters that won’t heal.

Resources

For further information, you can visit the following resources:

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