Epidermolysis bullosa is a terrible disease. Children who suffer from it, compared with butterflies because of the fragility of the skin. This disease can be congenital and acquired, in which numerous blisters form on the skin with bloody or transparent contents. At first they begin to get wet, and then turn into non-healing erosion. Such a disease is incurable and often fatal.
What is epidermolysis bullosa?
The concept of epidermolysis bullosa implies a whole complex of skin diseases of a genetic nature. With this disease, bubbles form on the mucous membranes and skin. They appear even with minor trauma or pressure on the skin. In simple terms, the skin of a kid with epidermolysis bullosa resembles very delicate butterfly wings.
This is due to the fact that in sick children between the outer and inner skin layer there are no special fibers arising from the interaction of proteins. People call this disease “butterfly syndrome”. Most often, it has a congenital character, triggered by mutations in the genes. With this disease, children may not even live to the age of three.
The main manifestation of the disease – bubbles on the skin. They appear as a result of the force action on the skin (shock, friction), but usually there is enough light touch. The child can not wear clothes, because even natural and seamless fabrics, in contact with fragile skin, lead to the formation of bubbles.
There are blisters on the skin and mucous membranes of the oral cavity, digestive tract, which is why the intake of coarse, non-rubbed food can cause injury. In extremely severe cases, babies cannot even drink milk because of severe pain, dying “starvation” death.
Bubbles are filled with bloody or clear contents.. They are very painful, but when they open, the liquid is poured out, the pain is reduced, and they themselves heal.
The term epidermolysis bullosa includes several forms of pathology, characterized by the course, manifestations and prognosis.
Simple congenital form
This disease is considered less dangerous and is passed on from generation to generation.. Butterfly disease is diagnosed immediately after the birth of a child or a few days later, and the boys are sick more often than girls.
The simple form is manifested by bubbles filled with serous fluid. They are localized on the skin areas subjected to pressure or friction. The main traumatic factor is childbirth. Bubbles in butterfly children occur in places that are in direct contact with the birth canal. Rashes range in size from a few millimeters to several centimeters. Neighboring skin areas remain unchanged.
Most often bubbles appear:
The mucous membranes are also affected, but much less frequently. Completely healthy are the nails. Bubbles open naturally or artificially. When they disappear, after them there are no defects on the skin, but temporary pigmentation may be present. With this form of the disease, the child develops normally.
This is a more dangerous form of butterfly syndrome. It arises a few days after birth. Bubbles can appear completely spontaneously, sometimes they are filled with bloody contents. When they open, they lead to the formation of scars or ulcers. Skin and mucous membranes, as well as nails are affected. Child development only in some cases remains normal.
Quite rarely, in case of a dystrophic form, rejection of body parts is observed. Particularly affected phalanges. Hypotrichosis and dental anomalies may develop. Children with this diagnosis cannot withstand infectious diseases.
This is the deadly form of butterfly disease.. It is diagnosed immediately after the birth of the baby and is characterized by the presence of bleeding wounds all over the body. There are bubbles on the mucous membranes of internal organs. Usually, the child immediately becomes infected with pneumococcal infection, which leads to sepsis and death.
Is it possible to cure this pathology?
Butterfly syndrome can not be cured, but with a simple form, you can hope for a favorable outlook. In this case, doctors and parents should provide symptomatic care to the baby. It lies in the fact that the treatment of the affected skin is carried out to prevent purulent complications. They can pose a serious danger to a child, especially a very small one.
Before you put on any clothes, even underwear, your baby is tied up with special bandages that do not stick to the skin. This is a very lengthy and painstaking process, sometimes taking several hours. In severe cases, dressings are performed 2 to 3 times a day. In this case, be sure to use a special moisturizer. It is vital for children with very thin skin.
External care for the skin of a baby butterfly is as follows:
- bubbles open on both sides with a sterile needle;
- affected areas are treated with antiseptic preparations, including Fuchsin and Eosin;
- if erosions occur, apply heliomycin ointment or antibiotic-based products.
A good result when taking care of a child with butterfly syndrome shows physiotherapy.
The disease appears immediately after birth.. Even during the passage through the birth canal of sick children skin is removed, like a snake. Most often, newborns, especially those with severe pathology, die in the first days or weeks of life.
Butterfly children are no different from their peers in physical and mental development. Although the disease lays its footprint, while creating a safe environment and ensuring good care of the child, you can protect from excruciating pain and make his life a little more comfortable. The critical period is the age of up to three years, when the baby is not yet aware that he cannot crawl or rub his cheeks or eyes. When the child matures, the skin begins to turn rough, becoming more stable, but never reaching the strength of the skin of the average person.
Prognosis for the diagnosis of epidermolysis bullosa usually unfavorable, because the disease is very rare, and doctors have practically no experience in treating such patients. A relatively favorable prognosis for life and health is possible only with a mild form of the disease. But not all parents can provide full-fledged care for their child, and often they themselves aggravate the situation by self-healing and treating wounds with phytotherapeutic drugs, bluing, homemade ointments and other similar means.
After such a “treatment”, the costs associated with the restoration of the baby’s epidermis are greatly increased. Wounds that appear on the skin can become infected and suppurate, resulting in death. Therefore, for children with epidermolysis bullosa, any skin injury can be fatal.
In this way, epidermolysis bullosa is a very dangerous disease, often fatal. The child suffers greatly, because of the non-healing ulcers on the skin, he cannot even wear clothes. If there is a genetic predisposition to this disease, then parents need to take a special responsibility to the issue of children’s planning.