Ихтиоздың сирек кездесетін клиникалық түрі - бір жақтық ихтиозформалы эритродермия: Клиникалық жағдай

Abstract

Congenital ichthyosis is one of the hereditary dermatoses that develop due to a violation of the keratinization process. The clinical manifestation of ichthyosis is characterized by dryness of the skin during physical examination and scaling of different degrees of clarity. Congenital ichthyosis can be classified as one of the most difficult and urgent problems in the field of dermatology and pediatrics due to the fact that the pathogenesis of this pathology is not fully studied, its persistent course, and the difficulty of achieving positive treatment results.In addition, despite many common features, types of ichthyosis are distinguished based on clinical and histological appearance: simple ichthyosis, recessive X-doubled ichthyosis, classic lamellar ichthyosis, bullous and non-bullous congenital ichthyosiform erythroderma. In all these types of ichthyosis, there is a violation of cellular kinetics and their physiological exfoliation process, resulting in excessive keratinization of the skin in the primary manifestation. During ichthyosis, there is a violation of the barrier function of the skin, loss of water between the epidermis and inability of the skin to retain moisture. In this article, we have shared a patient with these symptoms who was diagnosed with unilateral ichthyosiform erythroderma, one of the rare forms of ichthyosis encountered in our practice. This article is informative for general practitioners, neonatologists, pediatricians, dermatovenerologists and geneticists.