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

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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 (2000–10 000 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.

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About the authors

Stanislava Yu. Petrova

Joint-stock company Pharmaceutical enterprise «Retinoids»;
Mechnikov Research Institute of Vaccines and Sera.

ORCID iD: 0000-0003-3034-0148
SPIN-code: 7268-6944

PhD of Medical Sciences, the opinion leader of the medical department of Joint-stock company Pharmaceutical enterprise «Retinoids»opinion;  Senior Researcher at the laboratory of allergens

Russian Federation, 5a Maly Kazenny Pereulok, 105064, Moscow; 1a Svobody str., Ceramic microdistrict, 143983, Moscow region, Balashikha

Vera I. Albanova

Moscow Regional Research and Clinical Institute (MONIKI)

Author for correspondence.
ORCID iD: 0000-0001-8688-7578
SPIN-code: 5548-5359

Dr. Sci. (Med.); Professor of the Department of Dermatovenerology and Dermatooncology

Russian Federation, 61/2, Shepkina str., 129110, Moscow


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Supplementary files

Supplementary Files
1. Fig. 1. 6 years old girl. Vulgar ichthyosis: а — marked desquamation of the legs; б — folding and deepening of the skin lines on the palms. Author's observation. The informed consent of the child's parents to the publication of his image has been obtained

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2. Fig. 2. 26 years old man. X-linked recessive ichthyosis: а — small-scale peeling of the back; б — dark scales on neck, face free from scaling. Author's observation. The patient's informed consent to the publication of his image was obtained

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3. Fig. 3. 26 years old man. Lamellar ichthyosis: а — large saucer-shaped scales on the back; б — pronounced dryness of the skin, medium and large-scale peeling; в — deformation of the auricles, peeling of the face and scalp, sparse hair; г — peeling of the face, ectropion. Author's observation. The patient's informed consent to the publication of his image was obtained

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4. Fig. 4. 1 year old boy. Non-bullous congenital ichthyosiform erythroderma: а — erythroderma, finely scaled peeling, larger scales on the hands; б — erythroderma, finely scaled peeling on foot. Author's observation. The informed consent of the child's parents to the publication of his image has been obtained

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5. Fig. 5. 6 year old girl. Bullous congenital ichthyosiform erythroderma: а — horny layers of gray color on the body; б — horny layers on the hands, areas of apparently healthy skin in places where there were blisters before; в — horny layers in the form of concentric clusters on the elbows; г — massive horny layers on the feet. Author's observation. The informed consent of the child's parents to the publication of his image has been obtained

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