Pathogenetic mechanisms of transcutaneous sensitization to food allergens in inherited epidermolysis bullosa.

Congenital epidermolysis bullosa is a various group of inherited dermatoses resulting from a pathogenic variant of the genome encoding proteins of the dermo-epidermal junction. This disease is characterized by an early onset, continuous course with frequent exacerbations. Epidermolysis bullosa is clinically and genetically heterogeneous, includes phenotypes with different severity levels and is characterized by changes in 29 different genes. Classical forms of epidermolysis bullosa are divided into four main types depending on the level of bubble formation in the basement membrane zone. One of the most common symptoms in all subtypes of epidermolysis bullosa, as well as significantly reducing the quality of life, is itching. It occurs in areas with blisters or wounds or manifests as a generalized phenomenon, thus affecting both intact skin and healing wounds. The mechanisms of itching in epidermolysis bullosa have not been fully studied. It is likely that skin inflammation secondary to a violation of the skin barrier, cascades of wound healing and unregulated activation of epidermis-sensitive nerve endings are involved in its pathophysiology at the molecular and cellular level. Understanding the causes of these mechanisms is crucial for developing optimal treatment tactics for children with epidermolysis bullosa and improving their quality of life. This review summarizes updated data on clinical and genetic aspects of congenital epidermolysis bullosa.