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Epidermolysis bullosa, also known as butterfly skin, It is a rare genetic disease, but a very painful childhood skin disease that can affect and condition the lives of babies from birth.
In the epidermolysis bullosa, The proteins that make cement between the cells of our skin to maintain their integrity and structure are altered or are not. This causes a very fragile skin, similar to the wings of a butterfly, hence its name.
As they have this delicate skin, the minimum daily friction (walking, scratching, a hug ...) or sudden changes in temperature can cause blisters and wounds, and these injuries can be very painful.
It is a hereditary disease in most cases and is usually present at birth, although it is also possible that if it is a mild form it may appear later, when the child begins to crawl. It must be clarified that, in no case, is it a contagious disease.
The severity of symptoms is highly variable, from mild to fatal. Wounds heal very slowly, sometimes they may take months or even years, or they may never heal. Blisters can also appear inside the mouth, causing difficulty in swallowing or eating, around the eyes or nose or in other organs (esophagus, digestive tract, urinary tract ...).
There are different types of epidermolysis bullosa or butterfly skin disease depending on the depth to which the skin is affected. They are mainly divided into three:
It is the most common type. In the mild form, the blisters usually appear only on the baby's hands and feet and over the years they stop blistering. In severe cases, the blisters can be generalized and even appear inside the mouth, although it also usually improves with age. In this kind of epidermolysis bullosa, the blisters heal without scarring.
In mild cases, blisters appear on the hands, feet, knees, and elbows. In severe cases they can be generalized and appear in other organs such as the esophagus. In this case, as the blisters heal, they leave a scar, in such a way that if you repeatedly have scars in the same area (for example on the fingers) the skin can fuse. If this occurs in the toes it can cause difficulty walking. They are also at higher risk of having skin cancer.
In the mild case, the blisters appear on the hands, elbows, knees and feet of the newborn and improve by 2 or 3 months of age. In very severe cases, the child usually dies before the first year of life.
The diagnosis of this disease is made by a skin biopsy (a small cut in the skin to obtain a sample for analysis), and is confirmed by genetic testing. If there is an affected person in the family, genetic counseling is recommended.
Nowadays there is no cure for butterfly skin. Treatment consists of relieving symptoms, controlling pain, healing wounds, and preventing complications that may appear, such as joint deformities, skin infections, eating problems, malnutrition, anemia, eye problems or mobility problems. ...
In any case, the treatment must be individualized for each patient. On many occasions they must wear extensive bandages to avoid injuries, put pads on pressure areas and very meticulous care to heal wounds, similar to the care received by patients with large burns.
In relation to prognosis, mild forms tend to improve with age, but more severe types can lead to significant disability. However, we must rely on ongoing research to find a more effective treatment soon.
You can read more articles similar to The painful butterfly skin disease in babies and children, in the category of Children's Diseases on site.