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The disease can manifest itself at different ages. The child becomes ill in the womb.
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Most often, he is either already born with signs of ondansetron 8mg pills, or they appear in the near future after birth. In all cases, it is more correct to call the disease congenital epidermolysis bullosa. How many people get epidermolysis bullosa? Most often, the disease is recorded in children aged 1 to 5 years. Data from the National Epidermolysis Bullosa Registry, which is maintained in the United States of America, indicate that 1 in 50,000 newborns suffer from the disease. Over the 16 years of its existence in the United States, 3,300 people with this disease have been identified.
- In USA, epidermolysis bullosa affects 1 in 30,000 newborn babies. In Japan, the lowest prevalence of the disease, it is detected in 7.8 out of 1 million children born.
- The cause of this serious illness is mutations in the genes that are responsible for the synthesis of structural skin proteins. As a result, its cells lose strong connections with each other. The slightest external influences contribute to damage to the skin.
- The human skin consists of several layers. Upper - the epidermis is represented by keratinocytes. They constantly divide and, as they grow, move from the underlying layers to the upper - the stratum corneum, providing renewal of the skin and its protection.
- Keratinocytes are connected to each other by special bridges - desmosomes, from which protein filaments protrude - tonofibrils. The cells of the lower layers of the epidermis are also connected by the protein laminin.
- The epidermis is followed by a layer called the dermis. It includes collagen, elastic and reticular fibers, which are penetrated by numerous blood and lymphatic vessels, nerves, sweat and sebaceous glands, hair follicles. The dermis contains fibroblast cells. It is they who produce all the fibers that are part of buy ondansetron for sale.
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A deficiency of certain proteins involved in the structure of the skin is also possible: collagens, keratins, laminin and others.
Different clinical forms of epidermolysis bullosa are based on structural disorders of the skin, when, as a result of a mutation, the growth and maturation of damaged keratinocytes, the absence or reduction of retaining and fastening protein filaments occur. As a result, the bonds between the layers of the skin and its cells are weakened, and at the slightest impact from the outside, damage occurs with the formation of blisters. Classification of bullous epidermolysis.
Later, another group of the disease was identified.
Since the development of technologies that allow us to determine the structure of the skin at microscopic levels and determine its smallest structures, epidermolysis bullosa has been divided into 3 groups. In the modern medical community, the classification of the disease consists of 4 main groups and 6 subgroups. The subgroups collected different clinical forms of the disease. They differ in the type of zofran pills, microscopic changes in the skin, clinical manifestations, severity, prognosis.
So, epidermolysis bullosa can be simple, borderline, dystrophic.
A separate group stands out Kindler-syndrome. Simple epidermolysis bullosa includes suprabasal and basal. Forms of borderline epidermolysis bullosa can be localized and generalized. Dystrophic epidermolysis bullosa is dominant and recessive. Kindler syndrome is not divided into subgroups.
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Manifestations of bullous epidermolysis. Blisters, erosions of various sizes on the skin and mucous membranes are the main symptom of epidermolysis bullosa. Tamsulosin appear due to a decrease in the resistance of the skin to various influences from the external environment. Often this happens when the ambient temperature, pressure and friction change. Due to the fact that the skin has an unusual structure, bubbles appear, then erosion. Their healing in some types of bullous epidermolysis can take place with the formation of rough scars.
Babies with simple epidermolysis bullosa are born with blisters on the skin, or they appear in the first months of their life. Bubbles can be seen on the hands, feet, elbows, knees, shins, scalp. In the oral cavity, there are few or none at all. Blisters and erosions are painless and heal quickly.
Simple epidermolysis bullosa.
Nails do not change. If their detachment occurs, they are necessarily restored.
As the child grows, they will form less and less.
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In borderline epidermolysis bullosa, the changes concern the basement membrane of the epidermis. It is divided into generalized and localized. Their common feature is the appearance of blisters on any part of the body with extensive lesions. Changes in tooth enamel are also characteristic, it becomes thinner, point depressions appear on the surface of the tooth. They are often subject to caries. Borderline epidermolysis bullosa is more severe than simple epidermolysis bullosa.
Borderline epidermolysis bullosa.
They cover the scalp, legs, perineum, chest of the child. They rarely appear on the hands and feet, unlike other types of epidermolysis bullosa. The exception is the terminal phalanges of the fingers, on which the nails are located. The plates themselves are destroyed, peeled off and lost forever. Often all mucous membranes are affected by rashes.Â
Generalized moderately severe borderline epidermolysis bullosa differs from the previous version in a milder course. Bubbles can also be found in a newborn baby, but when they heal, scarring does not form. A distinctive feature of this clinical variant is focal hair loss and severe atrophy of the skin of the scalp. These babies grow and develop in accordance with their age. Illness has nothing to do with it. Dystrophic bullous epidermolysis.
This group of zofran bullosa is classified into two subgroups depending on the type of inheritance: autosomal dominant or autosomal recessive. This means that the genetic mutation is clearly inherited. In the first case, only one mutant gene, which is present in the father or mother of the child, is sufficient for the development of the disease. In the second case, both parents must be carriers of the defective gene, but they themselves will be healthy. But the baby will be born already with a serious illness.
Recessive dystrophic epidermolysis bullosa is more likely than other forms of the disease to lead to severe disability, although its severity may not be as severe.
