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Strategies for Differential Diagnosis and Management of Ichthyosis in Newborns

https://doi.org/10.31146/2949-4664-apps-2-2-55-64

Abstract

Congenital ichthyosis represents agroup of heterogeneous inherited skin diseases characterized by generalized keratinization disorder in the form of hyperkeratosis, caused by abnormalities in epidermal differentiation. The neonatal period is crucial for patients with ichthyosis due to the risk of developing complications, comorbidities, and fatal outcomes. Most complications arise from impaired skin barrier function. This article discusses the manifestations of ichthyosis in newborns, describes risks and complications, and suggests strategies for monitoring and treating patients. Purpose of the study: to present contemporary data on differential diagnosis and treatment strategies for newborn patients with congenital ichthyosis. Materials and Methods: review of Russian and international sources dedicated to the etiology, diagnosis, and treatment of ichthyosis was conducted. Results: Differential diagnosis of ichthyosis in the neonatal period is acrucial task, hence the most life-threatening subtypes are considered. The most severe progression with ahigh risk of fatal outcomes is characteristic of Harlequin ichthyosis. Collodion baby presents with less pronounced symptoms, is not afinal diagnosis, and may later develop clinical manifestations typical of ichthyosiform erythroderma, lamellar ichthyosis, or self-resolving congenital ichthyosis. Netherton syndrome is characterized by acombination of congenital ichthyosis with structural abnormalities of the hair shaft and apropensity for atopy. Epidermolytic ichthyosis, accompanied by the formation of blisters and erosions on the skin, requires differential diagnosis not only with other forms of ichthyosis but also with bullous epidermolysis, staphylococcal scalded skin syndrome, and toxic epidermal necrolysis. Every newborn with described manifestations requires support from amultidisciplinary team of specialists and timely therapeutic and diagnostic measures aimed at preventing the development of complications and deterioration of the patient’s condition. Conclusion: An analysis of global literature data and the practical experience of the authors indicate the complexities of differential diagnosis of ichthyosis forms in the neonatal period. To improve skin condition, prevent infectious complications, and correct electrolyte and respiratory disturbances, symptomatic treatment should begin before adefinitive diagnosis is established.

About the Authors

Yu. M. Shokurova
Charitable Foundation «BELA. Butterfly Children»; City Dermatovenerologic Dispensary
Russian Federation


O. S. Orlova
Reasearch Clinical Institute of Childhood of the Ministry of Health of the Moscow Region; National Medical Research Centre for Children’s Health; Charitable Foundation «BELA. Butterfly Children»
Russian Federation


N. M. Marycheva
Charitable Foundation «BELA. Butterfly Children»; Moscow Scientific and Practical Center of Dermatovenerеоlogy and Cosmetology; National Medical Research Center for Obstetrics, Gynecology and Perinatology named after AcademicianV. I.Kulakov
Russian Federation


A. A. Kuratova
Charitable Foundation «BELA. Butterfly Children»
Russian Federation


V. S. Polenova
Charitable Foundation «BELA. Butterfly Children»
Russian Federation


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Shokurova Yu.M., Orlova O.S., Marycheva N.M., Kuratova A.A., Polenova V.S. Strategies for Differential Diagnosis and Management of Ichthyosis in Newborns. Archives of Pediatrics and Pediatric Surgery. 2024;2(2):55-64. (In Russ.) https://doi.org/10.31146/2949-4664-apps-2-2-55-64

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