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The prognosis and life expectancy of a patient with epidermolysis bullosa depends on many factors - the form of pathology and its subtype, the quality of care, the presence of complications and the general condition of the patient. Epidermolysis bullosa simplex and some localized forms of viagra pill types have the most favorable prognosis. Sometimes the manifestations of the disease regress, and patients live a normal life. Achieving a stable remission, they never forget about preventive measures and follow all the recommendations of doctors.

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The most unfavorable prognosis for the dystrophic form of the disease. Multiple complications often lead to death in childhood.

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Simple congenital epidermolysis bullosa was described in 1886 by Koebner.

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The disease begins at birth or shortly thereafter. In places subject to mechanical irritation (friction, pressure, bruising), thin-walled blisters ranging in size from a pea to a walnut appear, with serous contents. Inflammatory changes in the circumference of the blisters are absent. They are located in the area of the knee, elbow joints, ankles, hands, scalp. For newborns with any kind of congenital epidermolysis, it is typical that the act of childbirth itself is the first mechanical injury, which leads to the appearance of blisters in the child in places that are in close contact with the mother's birth canal (head, limbs, buttocks, etc.).

 

After an independent (or artificial) opening of the blisters, the formed erosions quickly heal without atrophy and scarring, but with temporary pigmentation.

Very rarely (in 2-3% of patients) there is a lesion of the mucous membranes. Nail plates do not change. In 25% of patients, palmar-plantar hyperhidrosis is detected. The general condition of the patients is not disturbed. The course of the disease is mild, especially in girls. The mental and physical development of the child does not lag behind. An exacerbation of the disease is observed during periods when the child begins to crawl and then walk, as well as after warm baths and in the summer. Remissions occur in winter and become more frequent by puberty.

Localized, or summer, form of simple epidermolysis (Weber-Cockane syndrome) begins in the 1st or 2nd year of life, sometimes in adolescence, is transmitted in an autosomal dominant manner.

Bubbles are localized exclusively on the palms and soles, their tires are thicker, the contents are serous, often serous-hemorrhagic. Often revealed hyperhidrosis of the palms and soles. Exacerbations are observed only in the hot season, after warm baths, when wearing warm shoes. Dystrophic congenital bullous epidermolysis. The hyperplastic variety is transmitted in an autosomal dominant manner.

The disease is noted from birth or after a few days. Bubbles occasionally occur spontaneously, and usually occur with minor mechanical irritation. For example, even when a child himself hits a trinket toy several times on the same area (knees, feet, hands, stomach), hyperemia appears after a few hours, and then blisters with serous or serous-hemorrhagic contents, after healing of which scarring may remain. atrophy. Nikolsky's symptom is negative. Leukoplakia are often seen on the mucous membranes. The general condition is not disturbed, mental and physical development does not suffer. Hair and teeth do not change. Some children have dry skin, hyperkeratosis, and hyperhidrosis of the palms and soles.

The prognosis is favorable, the course of the disease improves significantly with the onset of puberty. The polydysplastic variety of dystrophic epidermolysis bullosa is inherited in an autosomal recessive manner and is one of the most severe forms of all epidermolysis bullosa. The disease begins at birth in the form of widespread blisters on the skin and mucous membranes, which can appear in large numbers not only after an injury, but also spontaneously. They have serous, but more often hemorrhagic contents.

Often there are various dysplasia in the form of xeroderma, hypotrichosis, acrocyanosis, endocrinopathies, dental anomalies (which are prone to rapid caries).

After opening, slowly healing erosions and ulcers are formed. Lesions are localized not only on the limbs, but also in other places, sometimes capturing significant surfaces (back, chest, abdomen, limbs). Nikolsky's symptom is positive. Significant itching is often noted. The nail plates atrophy very quickly and disappear altogether. The re-formation of blisters and ulcers, followed by scarring, leads to the formation of contractures, and sometimes mutilations, when disfigured stumps remain instead of hands or feet. Epithelial cysts (milia) are visible on the scars.

There may be ulcers of the small and large intestine, gallbladder, urinary tract stenosis, causing urinary retention, bladder hypertrophy and hypernephrosis.

Often there are severe lesions of the mucous membranes of the oral cavity, cicatricial shortening of the frenulum of the tongue, lesions of the larynx, bronchi, esophagus, anus, leading to stenosis, scarring, and sometimes perforations. Possible adhesions of the conjunctiva (sinblefaron), erosion of the cornea of ​​the eyes. The general condition of patients is severe, fatness is sometimes sharply reduced, anemia is observed, a significant lag in physical, and in some children, mental development.

Reduced resistance to infections (any kind of bullous epidermolysis is often complicated by a secondary pyococcal infection).