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Epidermolysis Bullosa (EB): Children with Fragile Skin

By HERWriter
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Skin, Hair & Nails related image Photo: Getty Images

People call them “butterfly children” because their skin is so easily damaged. Even mild traumas can cause painful blistering. Epidermolysis bullosa (EB) is a rare inherited skin disorder that impairs the body’s ability to make collagen and keratin. It is believed to affect one out of every 50,000 births. There are approximately 10,000 adults and children in the U.S. who suffer from EB. The majority of them have EB simplex, which causes blistering on their feet and hands.

There are three main forms of EB. Each has different symptoms and varying degrees of severity as well as sub-types that exist within each form. A person born with one form of EB will not go on to develop one of the other forms.

• Simplex EB-blisters occur primarily on the feet and hands in response to friction. Walking even short distances can set off the formation of blisters. The condition is worse in hot weather and blisters may also develop in the mouth or on other skin surfaces from tight clothing.
• Dystrophic EB-involves generalized blistering all over the body and webbing deformities of the fingers or toes as well as nails malformations. Severely affected individuals have eye involvement, growth retardation and anemia.
• Junctional EB-is a severe form of EB that also affects the skin internally in the body along the GI, urinary and respiratory tract. Infants born with junctional EB often do not survive due to overwhelming infections from opened blisters. People with junctional EP also can develop deformities of their fingers and fingernails and alopecia may occur.
All people with EB have impairments in how the layers of their skin are anchored together. The thin outer layer of the skin, the epidermis and the middle layer, the dermis are normally connected to each other with collagen filaments and anchoring fibrils. People with EB inherit from either one or both parents a genetic defect that impairs their body’s ability to make these protein-based connections.

The main focus for EB patients is the care of their wounds and prevention of infection.

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We value and respect our HERWriters' experiences, but everyone is different. Many of our writers are speaking from personal experience, and what's worked for them may not work for you. Their articles are not a substitute for medical advice, although we hope you can gain knowledge from their insight.