Congenital ichthyoses are a heterogeneous group of hereditary cornification disorders of varying severity, characterized by generalized desquamation, hyperkeratosis, and often inflammation of the skin. Ichthyosis is caused by various mutations in more than 50 genes encoding structural proteins and enzymes of the epidermis involved in maintaining and regulating the skin barrier function. Violation of the structure of the epidermis leads to increased transepidermal water loss, increased xerosis and desquamation. Currently, there are no specific treatments that can completely cure a patient with ichthyosis. Various topical agents are used to reduce dryness, flaking, hyperkeratosis, and transepidermal water loss. In recent years, scientific advances have changed the understanding of the pathogenesis of ichthyosis and made it possible to take a different look at approaches to therapy using anti-inflammatory biologics, small molecules and gene replacement therapy. The review considers the mechanism of the pharmacological action of drugs used in the treatment of ichthyosis, as well as presents authors’ own data on the use of innovative methods for the treatment of severe forms of ichthyosis in children.

Keywords: molecular therapy, autosomal recessive, ichthyosis