Skinless

There are two forms of the disease: acquired, ie, developed in the process of life, and hereditary, which is quite rare. Hereditary form of the disease can be transmitted both in the autosomal dominant and autosomal recessive type. There is a probability of inheritance recessively linked to the X chromosome. In their manifestations, these forms are similar, but acquired develops in adults, usually after allergic or immune reactions, infections.

The main symptom of this disease is the loss of skin elasticity, as a result of which it becomes flaccid, excessive, hanging down large folds. The skin is easily stretchable, but, unlike the changes in the Chernotubov-Ehlers-Danlos syndrome, it slowly returns to its original state. Changes are more pronounced on the face, but can be located throughout the body. Perhaps this development of the disease, when excess folded skin occurs only on the eyelids.

Disease occurs with the same frequency in persons of both sexes, exists from birth or develops in early childhood. In the X-linked form, the hooked nose, the enlarged upper lip and lobes of the ears, the presence of bony projections on both sides in the nape of the neck, increased joint mobility, and disorders of the digestive system( malabsorption syndrome) are characteristic. When ultrasound of the bladder is often revealed protrusion of its walls.

Pathological changes can affect only the skin or be systemic, manifested primarily by increased lightness of the lungs, cardiovascular anomalies, protrusion of the walls of the gastrointestinal tract and organs of the genitourinary system. The disease progresses constantly, often ending fatalities in early childhood due to pulmonary and cardiac failure, but with age, there may be a stabilization of the condition and even some improvement.

Treatment is aimed at improving lung and cardiovascular function, trophic tissue. In the early stages of the development of this disease, it may be useful to use penicillin, small doses of hormones, as well as drugs of copper in case of its deficiency. After stopping the progression of the process, plastic surgery is possible.