The possibilities of using retinol palmitate in the systemic treatment of generalized hereditary keratinization disorders

Hereditary ichthyosis is a group of generalized hereditary keratinization disorders characterized by general dryness of the skin, peeling, hyperkeratosis and often erythroderma. These manifestations are caused by mutations in genes mainly involved in the formation of the skin barrier. Hereditary ichthyosis is divided into syndromic and non-syndromic. Nonsyndromic ichthyoses include: vulgar ichthyosis, recessive X-linked ichthyosis, autosomal recessive congenital ichthyosis, keratinopathic ichthyosis and other forms. At present, the best result for achieving clinical remission has been established with oral retinoids: retinol palmitate, isotretinoin (1st generation of retinoids) and acitretin (2nd generation of retinoids). The ability of retinol palmitate to regulate keratinization processes, strengthen the epidermal barrier and have an antioxidant effect is used in the treatment of generalized hereditary keratinization disorders. Medium and high therapeutic doses (2 000-10 000 IU / kg / day) are used in the treatment. The prescribed dose of retinol palmitate differs in various nosological forms of ichthyosis, and depends on the severity of the pathological process, the age and weight of the patient, which must be taken into account when prescribing therapy to obtain the best result. It should be noted that clinical manifestations mainly regress at doses that do not lead to the appearance of signs of toxicity of the drug. The methods of retinol palmitate treatment of ichthyosis and ichthyosiform erythroderma are described.