The increased vulnerability of the skin is a congenital disease, which in modern medicine is called congenital bullosa. It’s a genetic diagnosis, in which the dermis is formed by numerous vesicles with purulent, serous content. The first is the weeping wounds on his body that turn into non-healing erosion.
What is epidermolysis bullosa
The dermis and the epidermis is two layers of leather that are connected by special adhesive composition. Failing this, there is a serious pathology, which is popularly called the «butterfly syndrome». Disease BAE becomes increasingly inherent nature, provoked by mutations in the genes. Epidermolysis bullosa is an autosomal disease in which not all patients live until the age of three. In practice is extremely rare, especially in the symptomatic treatment depends on the form of the pathological process.
Congenital epidermolysis bullosa
After the appearance of a child into the world is the simple form characteristic of the disease. The newborn has a rash on the body, however, after opening and removal of the serous content of wounds heal without any damage to skin, nails. In rare cases there is extensive damage to all the mucous membranes. Congenital epidermolysis bullosa is complicated in summer or with increased activity of the baby. In adulthood suprabasally bullosa treated successfully with medication, the symptoms can disappear at the onset of puberty.
Acquired epidermolysis bullosa
This disease develops in adulthood, is accompanied by extreme soreness appearing blisters on the skin. At the PBE after opening the epidermis is characteristic of the zone is compacted, formed the connective education. Foci pathology become mucous membranes, digestive tract, skin, feet and hands. Difficult the disease is treatable, but curable by the intake of synthetic hormones. Find out the cause of acquired epidermolysis bullosa provides a positive momentum for a long period of remission.
Dystrophic epidermolysis bullosa
In RHEED a dangerous bubble is detected at birth, as they grow older the body bubble, the rash spreads throughout the body. Dystrophic epidermolysis bullosa can be found on the hands and feet, elbows and knees, while in the pathological process involved mucous membranes, internal organs of the digestive system. With the development of complications does not exclude the shortening of the limbs with subsequent fixation in this position. For human tissues, there is a risk of narrowing of the oral cleft, shortening of the bridle. Mutations occur in the gene COL7A1.
Simple epidermolysis bullosa
In the pathological process involved the protein альфа6-бета4-integrin. The disease has three distinct forms, among which Weber-Cockayne, Kebnera and Dowling-Meara. Simple epidermolysis bullosa is autosomal dominant type of inheritance, but it is also possible autosomal recessive transmission. When degenerative variant Cockayne-Touraine observed genetic mutation of collagen type VII, chromosome Зр21.
If it’s recessive dystrophic epidermolysis bullosa, the type of inheritance defined as an autosomal recessive genetic mutation of collagen type VII, chromosome SP. The autopsy guestarray bubbles does not cause the patient discomfort, it does not exclude remission without medical treatment. Threat to mutant KRT5 and KRT14.
Border epidermolysis bullosa
This is the border between simple and dystrophic form of the disease where pockets of pathology can become all skin. If it is border bullous bullosa, the danger lies in complications that are not associated with dermatoses. It may be progressive anemia, narrowing of the esophagus and the mouth slit, the germination of the tongue and decreased visual acuity. Poba has two subtypes, where the severe form is considered to be a subtype Gerlitze with high risk of mortality. Mutations in epidermolysis bullosa forms occur in the genes LAMB3, LAMA3.
With this diagnosis the child is born already with bubbles, which are localized on different parts of the body. These are the first signs of the disease, which eventually only worsen. The patient develops photosensitivity, pigmentation of the skin, atrophy of the upper layer of the epidermis. Kindler syndrome affects the nails, leaving the nail plates the scars. Among internal complications bullosa – a mass lesion of the esophagus, urogenital system.
Causes of epidermolysis bullosa
The disease Epidermolysis bullosa also called the butterfly disease, as the skin becomes overly sensitive, damaged even with a light touch, is subject to activity by external factors. Often the prerequisites to the development of typical illness becomes a genetic factor that is passed from biological parents or progresses spontaneously. To answer the question, how many live children-butterflies, doctors stipulate different terms, depending on the etiology and nature of the disease epidermolysis bullosa.
In adulthood, the pathology is easier, but the child up to three years with this diagnosis live easy. Prognosis and life expectancy of the patient, doctors determined individually, not the last role in this question plays the etiology of the pathological process. For example, in the simple form of the disease become objects of attack proteins the basement membrane and are proteins of the basal layer of the epidermis, as desmoplakin, placefiles-1. Until the end of the study the clinic physicians and failed, there are still many questions about the causes of epidermolysis bullosa.
Symptoms of epidermolysis bullosa
Before you purchase in a pharmacy any drugs, it is important to find out in details how does this disease occurs when the body on the autopsy bubbles. This group of diseases is characterized by the following symptoms of epidermolysis bullosa, which are in the adult organism and the baby is mostly chronic. This:
- the formation of small bubbles on the body;
- ulcerations, and scarring;
- hyperemic effect;
- the lesions of the mucous membranes;
- enamel hypoplasia;
Treatment of epidermolysis bullosa
To avoid sepsis, and the development of squamous cell carcinoma, require timely to conduct therapeutic procedures. External treatment of epidermolysis bullosa – is a part of the complex, since for the solution of such global health problems requires a comprehensive approach. The main goal is the reduction of bubbles, the final deliverance of the foci of pathology, without the formation of scars. It is important to perform immunofluorescence analysis, and then proceed with intensive therapy. In this case we are talking about such drugs:
- Treatment local application of antiseptics to reduce erosion, prevent highly undesirable infections and propagation of Russian Academy of Sciences.
- After opening the bubbles are supposed to drain the purulent contents, and then use heleomyzidae ointment. It is not excluded the imposition of bandages, but without the pressure of bandages on the wounds.
- With the development of sepsis and shock, the doctor prescribes antibiotics and anti-shock drugs, holds symptomatic treatment.
- After the suppression of seizures in order to prevent it is appropriate to perform irradiation with ultraviolet rays, external treatment.
- In complicated clinical pictures against genodermatoses doctor wants you to take these synthetic hormones, like Prednisone, to allow the effect of remission.
- The first appearance of ulcers in children, BELLA better not to run the pathology, is necessary immediately to ensure local aseptic effect with known microbicides.
- If there is a genetic predisposition to epidermolysis bullosa forms, biological parents is required with special responsibility to approach the issue of planning for children.
Video: children butterfly