Epidermolysis Congenital Bullous

Epidermolysis Bullosa Congenital (syn.: epidermolytic bullous erythema, vesicular epidermolysis, streptocerulosis, etc.) is a severe skin disease associated with impaired synthesis of the keratin component in the layer of basal cells of the skin or their differentiation. Less commonly, there is a deficiency of the signaling proteins SLIT-ROBO interaction between keratinocytes with the development of epidermal rupture as part of the general system of neuromuscular connections.

Treatment tactics depend on the severity of symptoms, the age of the patient, and the presence of concomitant diseases. Depending on this, steroid hormones for external and internal use, normal human immunoglobulin, various methods of psychotherapy and psychocorrection are prescribed, as well as selective immunosuppressants are used to control the disease. Thanks to the use of the latter, the patient is able to independently get rid of many symptoms.

Congenital epidermolytic bullous (pemphigus vulgaris) is a syndromic form of bullous epidermolytic dysplasia of the skin. The development of hereditary epidermolysis bullosa is caused by mutations in the genes encoding the cytoskeletal molecules of the basement membrane of the skin: desmoglein-1, desmogenin and PLA.

Type I. Hand-Schuller syndromes and dystrophic epidemic keratolysis. These defects are characterized by minimal keratin abnormalities causing a keratin defect