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Background. Congenital ichthyoses (CIs) are a heterogeneous clinical-etiological group of genodermatoses. Typical clinical symptoms of this disease, regardless of the form, are generalized erythroderma, peeling, itching, hyperkeratosis, severe structural and functional disorders of the epidermal barrier, other organs and systems. Patients have an extremely low quality of life due to changes in appearance, discomfort, constant disease symptoms. Thus far, there are no effective treatment methods for ichthyosis. That is why scientific search for new therapies is the topical issue in pediatrics and pediatric dermatology.Objective. The aim of the study is to examine the cell-mediated immunity state in patients with CI via assessment of the pattern of lymphocyte subpopulations in peripheral blood. The research was conducted to study the content of the main and small lymphocyte subpopulations in 86 patients with established diagnosis of CI aged from 1 month to 18 years. The diagnosis was made according to the clinical data and the results of molecular genetic testing. Comparative analysis of blood immunological indicators in children with CI and in patients with other immunemediated chronic dermatoses: atopic dermatitis (AD; n = 68) and psoriasis vulgaris (n = 55).Methods. The level of T lymphocytes, T helpers (Th), cytotoxic T lymphocytes (Tc), B lymphocytes, NK cells, Treg-cells (Treg), activated T helpers (Thact), Th17 lymphocytes in peripheral blood was evaluated via flow cytometry using monoclonal antibodies. Statistical analysis was performed via Statistica 10.0. Differences between the groups were assessed via Mann-Whitney non-parametric test, differences were considered significant at p < 0.05.Results. A significant increase of activated T-helpers level in peripheral blood was revealed in patients with CI and psoriasis compared to children with AD (p < 0.001), as well as an increased levels of B-lymphocytes and Treg in children with CI (p < 0.05).Conclusion. Children with CI have shown some features of cell-mediated immunity such as: pathological activation of Th lymphocytes, impaired terminal differentiation of naive CD4+ cells to Thact, Treg, Th17 lymphocytes and their proliferation. Comparative analysis of mentioned immunological indicators in children with CI, psoriasis and AD has shown comparable results of increased Thact lymphocytes levels in patients in CI and psoriasis groups. This results open up potential of using immunobiological drugs of psoriasis target therapy within the new management strategy for children with CI.

Background. The study of psoriasis biological therapy aspects in children has certain topicality due to the small number and disunity of individual observations and the lack of special registers for pediatric patients.Objective. Our aim was to study ustekinumab efficacy and safety in children with plaque (PP), erythrodermic (EP) and palmoplanar (PPP) forms of psoriasis.Methods. The analysis of ustekinumab efficacy and safety has been carrying out for 1 year. The evaluation of therapy efficacy was based on definition of improvement of PASI scores (PASI 75, PASI 90 and PASI 100) on the 16th, 28th, 40th and 52nd weeks of follow-up and children's dermatology life quality index (CDLQI). Ustekinumab therapy safety analysis was based on registration and evaluation of adverse effects. Results. The study included 67 children with PP, EP and PPP aged 12 to 18 years. PP group results: the PASI 75 response at the 52nd week of therapy was observed in 35 children (100%), PASI 90 — in 33 (94%), PASI 100 — in 30 (86%). EP group results: 10 patients (56%) have reached PASI 75 on the 16th week, while none of patients have improved to PASI 90 and PASI 100 scores. The PASI 75 response at the 52nd week of therapy was observed in 18 children (100%), PASI 90 — in 17 (94%), PASI 100 — in 7 (39%). Only 1 patient (7%) with PPP has showed the score decrease to PASI 75 on the 16th week. Adverse effects were reported in 2 patients.Conclusion. Ustekinumab is the effective and safe treatment for moderate and severe forms of PP and EP in children, and it can also be considered as one of the alternative methods for PPP treatment in pediatrics.

Psoriasis is multifactorial systemic disease characterized by excessive hyperkeratosis due to impaired keratinocytes proliferation and differentiation. This disease often manifests in childhood and it is usually associated with the development of comorbid conditions some of which are combined by the term «psoriatic march» (obesity, metabolic syndrome, etc.). The course of psoriasis and related comorbidities in children has several specific features that determine topicality of studying all the aspects of clinical diagnosis and prevention in childhood. The article summarizes recent ideas on the prevalence, pathogenesis features and early diagnosis of comorbid diseases in children with psoriasis.

Background. Nowadays, dermatoses with mixed clinical picture and resistant to classical management become more common. The presence of various genetic disorders typical for most chronic dermatoses may indicate possible combination of several nosologies.Clinical Case Description. The article presents the clinical case of multimorbid condition in 10 years old patient who has nucleotide variants in CARD14 and EXPH5 genes. Mutations in CARD14 gene are typical for patients with type 2 psoriasis and pityriasis rubra pilaris (autosomal dominant type), while mutations in EXPH5 gene are typical for patients with non-specific epidermolysis bullosa (autosomal recessive type). Mutation in the TGM1 gene that is described in patients with congenital ichthyosis (autosomal recessive type), pathogenic mutations in KRT74 gene typical for ectodermal dysplasia, hypotrichosis and uncombable hair syndrome, and mutations in the KRT86 gene typical for monilethrix were also revealed. Medical history taking and histological examination as well as clinical data evaluating are crucial for correct diagnosis. They allow to understand the absence of the such manifestations in relatives and reveal various pathological processes in the epidermis. Molecular genetic testing with new generation sequencing (NGS) helps to finally establish the diagnosis and determine the further tactics for patient management.Conclusion. Multidisciplinary approach and use of high-technology methods of examination and treatment (such as molecular genetic testing and biological therapy) are required for final diagnosis in severe forms of chronic dermatosis resistant to treatment and for decision on correct tactics for the further management of such patients.

Psoriasis is chronic inflammatory skin disease that can develop at any age. Approximately 20–30% of all patients report about first rashes before the age of 18. Psoriatic arthritis is one of psoriasis comorbid conditions. Its signs can range from mild to extremely severe destructive forms. Arthralgia, joint stiffness and swelling are the most common symptoms. Early psoriatic arthritis treatment onset allows to control joint damage which usually occurs during the first 2 years of the disease. The moderate and severe course of psoriasis and psoriatic arthritis may require systemic therapy, however, there is not much data on the efficacy and toxicity of systemic agents in the pediatric practice. This article provides the review of studies on etanercept efficacy and safety in children with psoriatic arthritis.