The blisters quickly merge with each other, and after they are opened, painful, poorly healing, bleeding ulcerative necrotic foci remain on the skin and mucous membranes. Nikolsky's symptom is positive. There are no scars or milia. There are congenital nail dystrophies, atrophic changes in the skeleton. The course of the disease is very severe, quickly complicated by pyococcal infection, sepsis, and ends fatally in the first months of a child's life. For the purpose of prenatal diagnosis of genodermatosis, methods of fetoscopic skin biopsy followed by light, and especially ultramicroscopic, research have been developed in recent years. A biochemical marker of recessive dystrophic epidermolysis bullosa is an increase in collagenase activity in fetal skin biopsy specimens - 3.5 times higher than in healthy cells.
Treatment due to the lack of specific methods is very difficult. In the period of cialis and early childhood, when the immune mechanisms are not yet mature enough, the prevention of the development of sepsis and its rapid and adequate treatment are especially important. The baby needs to be breastfed.
In severe forms of epidermolysis bullosa, glucocorticoid hormones most effectively prevent blistering. The administration of corticosteroids can save the lives of children suffering from a lethal form of the disease. For such children, prednisolone is first used - up to 40-60 mg per day, after the condition improves, the dose of the drug is gradually reduced until it is completely canceled. Anabolic agents are also prescribed (Nerobol, Nerobolil), parenteral administration of broad-spectrum antibiotics, infusion of tadalafil, plasma.
With exacerbations of polydysplastic and hyperplastic forms of epidermolysis, only short courses of glucocorticoid hormones at the rate of 1-2 mg / kg (calculated as prednisolone) orally for 10-15 days are advisable, followed by a gradual decrease in dose until the drug is completely discontinued. The use tadalafil pills small doses of prednisolone is effective in preventing the occurrence of blisters on the mucous membranes, strictures of the esophagus, the formation of syndactylia, eye and dental complications.
In many patients, especially with the hyperplastic form of cialis, a beneficial effect is observed when prescribing repeated courses (2-3 months with a break of 2-3 weeks) of tocopherol acetate, which restores the normal structure of the collagen and elastic fibers of the dermis. For the purpose of immunocorrective and restorative effects, sodium nucleinate, metacil, plasma infusions, gemodez, repeated courses of 10-12 injections of gamma globulin, vitamins A, C, B 1, D, rutin, nicotinic acid, pyridoxine, vitamin B 12, calcium pantothenate and pangamat, iron preparations. A. V. Smirnov (1986) obtained an improvement in the clinical course of the polydysplastic form of epidermolysis bullosa in two children (2 years and 6 years old) with intramuscular injection of Solcoseryl 2 ml daily for 10 days.
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It is necessary to systematically monitor the state of the blood in order to timely detect and treat anemia. To prevent the formation of contractures, it is important to ensure a full range of motion in all joints. In order to eliminate gum hyperplasia, the child should be taught to rinse the mouth regularly.
In dystrophic forms of the disease, the drug cyanidalon-3, which is a derivative of flavones and related to membrane stabilizers, is being studied. When using it, a calmer course of the disease was noted, the formation of blisters decreased, erosion healed better, which prevented the appearance of strictures of the esophagus, mutilation and syndactylia. This drug in vitro increases the resistance of collagen against the effects of collagenase.
External therapy: emerging blisters must be opened in two places with a sterile needle, lubricate the tires with a 1-2% aqueous solution of aniline dye (magenta, eosin, brilliant green, methylene blue).
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Lotions with tea are used, the crusts are smeared with olive oil. On erosion and ulcers, iruksol ointment, solcoseryl ointment, dermatol (3-5%), heliomycin ointment are regularly used, oil solutions of vitamins A, E, D, mixed in equal proportions, are applied. Ointments, creams and aerosols containing glucocorticoid hormones, antibiotics, sea buckthorn are also prescribed.
Bandages and patches should be avoided whenever possible to preventI friction, injury to the skin. A good effect is provided by general therapeutic baths with decoctions of oak bark, viburnum, chamomile, string, as well as with a solution of potassium permanganate, zinc sulfate and a bath with the addition of olive oil.