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The simple type (epidermolytic according to the Pearson classification of 1962) is the most common, occurs in 75% of cases and is characterized by the formation of blisters in the upper layers of the epidermis on the skin of the feet, hands, and in severe cases, the entire body.

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Divided into 2 main subtypes suprabasal, in which plakophilin-1, desmoplaktin, etc., becomes target proteins, as well as basal subtype, causing changes in the structure of alpha-6 / beta-4 integrin, and skin disorders can be localized, generalized (bubbles occur in groups) and in the form of tamsulosin pigmentation.

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The most common clinical forms are lethal acantholytic, superficial, withmuscular dystrophy, with pyloric atresia, ognassky, migratory annulare, etc. The borderline type (lucidolytic) leads to the formation of blisters in the region of the light plate of the basement membrane and occurs in the form of the Herlitz subtype, which disrupts the structure of laminins 332 and 5, as well as other subtypes involving not only laminins, but also type XVII collagen and α alpha-6 in the pathogenesis /beta-4 - integrin. In addition, the clinical form is inversion, with a late onset, in the form of laryngo-onycho-cutaneous syndrome.

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Dystrophic type (dermolytic) - affects the upper part of the papillary layer of tamsulosin pills, which is dominant and recessive subtype and develops due to a violation of the structure of the fourth type of collagen.

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The type of inheritance of epidermolysis bullosa occurs as autosomal recessive (most common), autosomal dominant, and uniparental disomy, and somatic mosaicism. Factors provoking congenital epidermolysis bullosa.

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This type is distinguished by the development of a pathognomonic symptom - the appearance of areas of abundant granulation tissue on various parts of the body (as in the photo of borderline epidermolysis bullosa), for example, localized symmetrically around the mouth, in the middle part of the face, around the nose, on the upper back, in the armpits or nail rollers. Pathology can lead to onychodystrophy and even complete loss of nail plates, a large number of Flomax, severe scars on the body, including scarring alopecia, crusts on the skin, ulcers in the oral cavity, enamel hypoplasia and severe dental caries.
Borderline epidermolysis bullosa.
Healing of blisters most often occurs without scarring, sometimes even resembling herpes simplex, but the likelihood of relapse is very high. Among the possible systemic complications, polyetiological anemia, growth retardation, erosion, skin vesicular rashes and detachment, and strictures in the gastrointestinal tract are usually detected. The risk of their development increases depending on the area, degree of destructiveness and depth of skin lesions.
Dystrophic bullous epidermolysis.
Borderline epidermolysis bullosa is characterized by extremely high mortality, mainly in the first years of life, caused by the cessation of weight gain, sepsis, pneumonia, or obstruction of the trachea and larynx. Extracutaneous manifestations of dystrophic BE. Biopsy plays the most important role in the diagnosis. Due to the availability of Flomax and polyclonal antibodies against proteins of different layers of the epidermis involved in the pathogenesis of epidermolysis bullosa, immunohistological methods are gaining more and more popularity today.

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The immunohistochemical method of research and the method of indirect immunofluorescence make it possible to determine the state of expression of structural proteins with hereditary defects in skin cells - keratinocytes and basement membranes (the basis of the epithelium, which performs a barrier and trophic function), as well as the distribution pattern of proteins in previously formed or artificially provoked blisters, including number — their depth of localization.

Thanks to modern methods of DNA diagnosticsIt is necessary to quickly classify the type of pathology, identify structural proteins that have undergone mutations and make a clinical prognosis. An innovative method of genetic analysis - direct sequencing makes it possible to identify mutations, their type and localization, and reliably confirm the diagnosis.

In addition, an important role is played by the collection of a family history and the patient's medical history, a comprehensive examination of the whole organism, and laboratory tests. Treatment of bullous epidermolysis.

Preventive and symptomatic treatment is considered to be the most effective and widespread today, since no radical therapy has yet been developed to cure any of the subtypes of EB, although an accurate diagnosis makes it possible to significantly improve the quality of life and reduce the likelihood of relapses. Epidermolysis bullosa is a rare and terrible disease in which the skin and sometimes mucous membranes are affected. Any careless touch hurts the baby and leaves wounds. Butterfly children suffer not only from their diagnosis, but also from the lack of knowledge of hospital staff. The child receives the first injuries in the maternity hospital. Sometimes they are even incompatible with life. Epidermolysis bullosa has many forms, but all of them are incurable.