Specificity of the condition of the skin barrier in patients with congenital epidermolysis bullosa as a factor of transcutaneous sensitization by food allergens

Abstract

Congenital epidermolysis bullosa is a heterogeneous group of hereditary dermatoses resulting from a pathogenic variant of the genome-encoding proteins of the dermo-epidermal junction. Epidermolysis bullosa is mainly manifested as the formation of blisters and erosions on the skin and mucous membranes in response to minor mechanical action. Itching is one of the most common symptoms of epidermolysis bullosa, reduces the quality of life, and causes additional skin damage. The influence of comorbid pathology, which can increase itching, is not excluded. Skin inflammation secondary to a disruption in the skin barrier, wound-healing cascades, and unregulated activation of epidermal sensitive nerve endings are involved in the pathophysiology of itching at the molecular and cellular levels. Diffuse damage to the skin and mucous membranes, leading to the loss of their barrier properties, contributes to the excessive intake of antigens, including allergens of food and non-food origin, and to transcutaneous sensitization. However, food sensitization and food allergy in these patients have not been sufficiently studied. Understanding the causes of these processes may be crucial for the development of optimized techniques for managing children with congenital epidermolysis bullosa and improvement of their quality of life. This review summarizes updated data on clinical and genetic aspects of congenital epidermolysis bullosa.