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The article analyzes the most significant genodermatoses associated with a high risk of allergic reactions that may occur in the practice of a dermatologist and pediatrician, such as ichthyosis and ichthyosiform dermatoses, Netherton syndrome and other ichthyosiform erythroderma, peeling skin syndrome, SAM syndrome, as well as congenital bullous epidermolysis. The article also describes in detail the pathogenetic aspects of transcutaneous sensitization, the development of food allergies and the listed above genodermatoses, two illustrative clinical cases are given.