Summary Ichthyosis (Greek "Ichthys" - fish) is a group of hereditary, in rare cases, acquired diseases from the group of keratoses, characterized by a diffuse violation of the process of keratinization of the epidermis and is manifested by dry skin and severe peeling, often resembling fish scales. We studied the dynamics of congenital ichthyosis in the Republic of Azerbaijan based on the results of a retrospective statistical analysis of the medical documentation of children's KVD No. 3 and RCVC 2021-2022. (average 9.2 per 1,000,000 population). 186 primary cases of congenital ichthyosis were identified, among which 134 (72.04%) were over the age of 18, 52 (27.96%) were in childhood and adolescence; including 153 (82.26%) males, 33 (17.74%) females.
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