Butterfly Syndrome: Bullous Epidermolysis

Bullous epidermolysis (BE), or, as it is also called, the butterfly syndrome is a very rare hereditary disease. Such children have been called children of butterflies. If you have some beautiful associations with this name, then the disease itself is far from aesthetic. In fact, patients with bullous epidermolysis spend their whole lives in painful suffering. The reason is their skin, fragile, like the wings of butterflies.

Unfortunately, the disease "butterfly syndrome" can not be cured. Because it is deeply embedded in the genetic level. Such children need special care: a completely soft environment, clothes and shoes. In Russia, this incurable disease is not even included in the list of the most rare diseases. However, there is a specialized fund for helping butterfly children "Bela".

Every year, a lot of money is donated to this fund, in order to somehow help children who have the disease "butterfly syndrome". Photos of patients can be seen in our article, and in scientific medical literature. However, information on bullous epidermolysis has not been properly disseminated in Russia and CIS countries.

The disease "butterfly syndrome" is not known to the general public. Few can even pronounce her complex name - bullous epidermolysis. However, there are such children all over Russia and the CIS countries. Each of the patients has the same problem: local doctors do not know how to deal with them, the diagnosis is made "by eye", and not always true regarding the types of this disease. And sometimes mothers generally hide such children, which leads to their death due to improper care.

The butterfly syndrome is characterized by the appearance of a bubble rash on the skin of a person. Depending on the vesicles, the following types of bullous epidermolysis (BE) are distinguished:

1. Plain. It is formed on the upper parts of the skin. The skin thickens on the palms and soles of the feet. Vesicles can form even in the mouth.
2. Frontier. Appears on skin-epidermal connections. In severe form of the disease, the vesicles can appear on the walls of the esophagus, intestine and in the genitourinary system.
3. Dystrophic. Vesicles appear on the knees, hands, elbows, mucous membranes, can affect the esophagus, cause growth and nutrition problems. This kind is also characteristic of the fusion of the fingers and toes.

Butterfly syndrome is the result of an incorrect formation of the protein in the skin or its complete absence. Spontaneous gene mutations can also cause the disease .

At birth, the skin of a sick child slides like a skin of a snake. Of course, this causes a shock reaction to the mother. Many simply refuse sick children in the maternity hospital. But the chance to survive in a child with a disease bullous epidermolysis in an orphanage is almost zero. Most of the children die with improper care. After all, these children need to be treated very carefully.

The most difficult period in patients is the age of up to 3 years. Gradually, with proper care, the skin becomes coarse. And young children, when they try to crawl, rip off all their knees and hands. The skin becomes blistered and peels off, causing severe pain. Such children can not even shake hands like an ordinary person. With growing up they gradually learn to protect themselves. At the age of more than 3 years, life becomes easier.

However, patients with a severe form of bullous epidermolysis experience severe pain and itching daily, from which even antihistamines do not help. In addition, every three days they must endure necessary and painful dressings. Such children, like all other terminally ill, are special. They have unimaginable eyes, probably from this unbearable pain, which we can not understand.