Butterfly Skin Syndrome ( Epidermolysis bullosa (EB))

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By Heather Walker BSc

This rare, inherited but also incurable genetic condition causes the skin to be so fragile that even the slightest touch can cause the skin to tear or blister.

Some children are named ‘Butterfly Children’ as their skin can appear as delicate as a butterfly wing. Any small injury can cause a reaction, even something as simple as rubbing, scratching.

Blisters are not restricted to the outer skin but can also show signs in places like the stomach or mouth. Most types of EB are inherited whether from one or both parents and symptoms can usually be seen in babies and young children although, symptoms can also arise in teenagers and young adults.

Mild forms can improve with age but most treatments are concerned with the care of and the prevention of new blisters.

Symptoms Include:

Skin that blisters easily (especially hands and feet), malformation of nails,extra-thick skin on palms of hands and soles of the feet, scarring, and hair loss caused by blistering, transparent skin, small white spots, badly formed enamel causing tooth decay, difficul ty swallowing and very itchy and painful skin.

Complications of epidermolysis bullosa can include:

• Sepsis: An infection of the bloodstream that spreads quickly in the body causing shock and organ failure.

• Fusion: Severe forms of EB can result in fusion of toes and fingers while causing extreme bending of fingers, knees, and elbows.

• Infection: Bacteria due to blisters.

• Nutritional problems: Difficulty eating caused by blisters in the mouth with malnutrition and anemia as a result.

• Skin cancer: due to the fragility of the skin.

• Death: Due to extreme blistering and the increased risk of infections.

Prevention:

Although there is no cure for EB there are certain preventive steps that can be taken to prevent blisters and possible infection, such as:

• Gentle handling of the sufferer, particularly children who need to be lifted.

• Maintain a cool environment.

• Moisturizing the skin.

• Soft clothing with no seams that rub.

• Stop scratching: Regular cutting of the fingernails can prevent scratching even while sleeping.

• Cover hard surfaces: Cushions and towels work best.

When to see a doctor?

If you or any of your family members develop blisters for no reason or have problems swallowing, breathing or find red, painful, or swollen skin or infection that appears to become infected that causes a pungent smell coupled with fever or chills. This condition may not be curable but with quick treatment and care, it can be livable with.

Seb
Author: Seb

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