Epidermolysis Bullosa

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Epidermolysis Bullosa

Epidermolysis bullosa

Epidermolysis bullosa is a section of unusual diseases which lead to blistering of the skin. The blisters may show up as a result of heat, or friction from rubbing, minor injury, scratching or adhesive tape. In severe cases, the blisters may appear in the internal parts of the body, such as the lining of the intestines or the oral cavity.

Most of the times, this condition is inherited. Usually the people who are most likely to get affected are infants and children in the age group of 5-7 years.

 What are the signs and symptoms?

  • Deformity or loss of toenails and fingernails
  • Fluid filled blister formation on the skin, particularly on the hands and feet as a result of friction
  • The skin of the palms and soles get thickened
  • Internal blistering, including the esophagus, vocal cords and upper airway
  • Thin-appearing skin (atrophic scarring)
  • Blistering of scalp, scarring alopecia (scarring and hair loss)
  • Tiny white skin bumps or pimples
  • Difficulty in swallowing (dysphagia)
  • Dental problems, like tooth decay from poorly formed enamel

Epidermolysis bullosa blisters generally do not appear until a toddler first begins to walk or until an older child begins new physical activities that more intense friction on the feet.

 What are the causes?

It is an inherited disorder and according to researchers’ findings, genetic involvement may be involved in causing it.

Below are the types of epidermolysis bullosa:

  • Epidermolysis bullosa simplex: This is the commonest form. Generally it begins at birth or in early infancy and the parts that are most likely to get affected are the palms and the soles.
  • Junctional epidermolysis bullosa: It is one of the severe forms and becomes apparent at birth. A baby affected with this disorder may develop a hoarse-sounding cry because of continual blistering and scarring of vocal cords.
  • Dystrophic epidermolysis bullosa: In this condition, the person experiences mild to severe signs and symptoms. Generally it becomes apparent at the time of birth or during early childhood.
  • Kindler Syndrome: It is a rare type and usually becomes apparent at birth or soon after.

This condition is called mixed type since blisters appear across the layers of skin.

 What are the risk factors?

A significant family history of epidermolysis bullosa is the major risk factor for developing the disorder.

 When to consult a Doctor?

In case your child or you develop unexplained blisters, consult a Dermatologist. If at all you notice any signs of infection in the area surrounding blister, like, redness, pus, increasing pain or warm skin.

 What are the different modes of treatment?

Medications like oral antibiotics may be prescribed in order to help prevent infection or sepsis. In case of dysphagia, oral anti-inflammatory drug (corticosteroid) may help reduce the pain.

Surgery may be thought of in order to correct normal motion or to improve your capacity to eat a healthy diet.

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