Good evening, dear readers. Today I brought you a very interesting and little-known subject: children with butterfly skin.

Butterfly skin, technically known as Epidermolysis bullosa or EB, is a genetic disease, rare and incurable to date.
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Epidermolysis bullosa, butterfly skin, is a genetic disease of very rare incidence in the population, which causes an important fragility of the skin and mucous membranes.

It is commonly known as "butterfly skin", because with any rubbing the dermis and epidermis is detached. For this reason the children wear bandages on a large part of the body, to protect the wounds from any scratching or contact with the outside.

It causes a lot of pain and the immediate consequence is a drastic decrease in the quality of life, both of the affected person and of their relatives and loved ones.

This condition is not detected in tests that are done during pregnancy and also has no cure.
One of the physical consequences of this disease is that it causes blisters in areas such as the esophagus and eyes, causing the swallowing canal to close and people can not eat.

According to the latest data from DEBRA International, it is estimated that the incidence of the disease is 15-19 affected births per million births. The estimated prevalence is 10 people affected per million inhabitants. In Spain there are approximately 500 people with the disease.
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But this disease goes far beyond the skin. It happens to some children, that their teeth fall out, their mouth closes and their fingers deform.

The knowledge about this condition is still very scarce, so in general the parents find out about the problem very late and unexpectedly and they must live the grieving process abruptly.

Complications continue as the children grow up because they must be given very strong painkillers before bathing, for example, because they do a lot of damage, suffer from fears and panic attacks.

In people with Butterfly Skin, the proteins that make it possible for the skin to have its characteristic resistance are absent or do not work correctly. That is, colloquially we say that the "glue" of the skin does not exist. Depending on the type of protein that is absent or functioning incorrectly, Epidermolysis bullosa affects each person differently.
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Evolution.

Patients with the most severe forms of the disease do not usually have a good evolution, since it drastically decreases the prognosis of life, so many die in the second decade of life as a result of developing skin cancer.

Hopes.

There is a treatment that is currently being developed in its experimental phase and offers an alternative to patients and their families.

It is a procedure with stem cells whose inoculation route is the blood and could help compensate the damaged skin, reduce pain, burning and itching caused by blisters and wounds.

The new research also seeks:

-Improve the knowledge of genetics and biology of all types of bullous epidermolysis, since a better understanding can bring us closer to the discovery of new diagnoses and treatments.
-To understand the nature of wound healing and the development of skin cancer in Epidermolysis bullosa, and to investigate the development of better treatments and prevention strategies.
-Work in the development of therapies, including possible genetic, cellular, medical or protein therapies.
-Support clinical care research to improve the management of the disease through the relief of symptoms.

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