REVIEW
Federation Skin lesions with development of erosive-ulcerative defects and impaired skin barrier function are common for large number of diseases. Successful epithelization of skin defects depends on the wound process, body’s compensatory functions, environmental factors and correctly selected treatment. Comprehensive treatment should include systemic and local therapy as well as current dressings. The article shows current dressings possibilities the treatment of various skin diseases, outlines all pros and cons of the major current dressings.
Atopic dermatitis (AD) is the disease with chronic inflammation, epidermal barrier dysfunction and microbial dysbiosis. AD is widespread, including pediatric population. The article discusses the disease’s pathogenesis: skin barrier deficiency, immunological causes of chronic inflammation, characteristics of normal skin microbiome and its disorders on both affected and unaffected skin of children with AD. Main principles of systemic treatment for moderate and severe forms of disease are considered. Features of targeted therapy with dupilumab (IL 4/IL 13 inhibitor) in children with moderate and severe forms of AD are discussed. The overview of the research results on the dupilumab efficacy and safety is presented.
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.
Vitiligo is a common skin disease characterized by idiopathic progressive skin hypomelanosis. Vitiligo is associated with several comorbid autoimmune diseases such as localized scleroderma. This article demonstrates the general development mechanism of these pathologies, as well as the key aspect of cross-effect between autoimmune diseases on the molecular level. Recently, dermatologists have noted the increasing number of patients suffering from combined pathologies. Such patients (in pediatrics) have torpid course of disease and no pathognomonic symptoms. That exaggerates the diagnostics and adequate therapy prescription. This leads to increased awareness among physicians of different specialties on possible combinations, clinical presentation and pathogenesis aspects of such conditions.
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.
Pruritus is one of the main clinical manifestations of atopic dermatitis, and it significantly reduces the quality of life of patients in childhood. Scientific images on its pathophysiological basis have now undergone significant changes. The histamine exceptional role in pruritus development was confounded, as well as data on immune system involvement in its maintenance was given. This article presents current data on differential approaches to pruritus management depending on its etiopathogenetic characteristics. The role of dermocosmetics in restoration of the skin barrier as the first stage prevention of pruritus in atopic dermatitis was considered. The results of clinical studies showing efficacy of topical agents (innovative component with anti-pruritic action — STIMU-TEX) application are presented.
CLINICAL OBSERVATIONS
Background. Anaplastic large cell lymphoma (ALCL) represents 15% of all non-Hodgkin lymphomas (NHL) in children, and it is characterized by aggressive course and involvement of various organs and systems (including skin) in the pathological process. Skin tumors at NHL in childhood are rare. Thus, cases of skin localization are more oftenly diagnosed at ALCL among all morphoimmunologic variants of NHLs. The description of any new case of skin lesions in patients with ALCL has undeniable interest and scientific and practical significance due to its extremely rare frequency.
Clinical Case Description. Patient G., 11 years old, has firstly noted inflammatory nodes on the skin of scrotum. The generalization of skin lesions on tibia, face, lymph nodes and bones was recorded due to late admission to the hospital. The inflammatory-necrotic process has been revealed at skin histological examination. Local antiseptic and antibacterial therapies were ineffective. The ALCL was diagnosed on morphoimmunologic re-examination of the skin. Life expectancy after disease manifestation to patient’s death was 6 months.
Conclusion. Rare cases of skin involvement at ALCL require mandatory histological and immunohistochemical examinations. Second biopsy with reconsideration of histological preparations in the reference center may be necessary due to clinical-laboratory picture of the inflammatory process accompanying the tumor itself.
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.
Clinical Case Description. The clinical case of Wells syndrome in female 4 years old patient is presented. Clinical findings included symmetrical skin lesions, nodes and large irregular edematous plaques of red-purple color with clear fluid vesicles on its surface. The disease had wavy course: rashes have recovered spontaneously over 7–10 days, new elements appeared alongside with feeling unwell, fever up to 37,8°C and abdominal pain. Similar clinical findings of rashes were observed in paternal relatives of the child.
Conclusion. Differential diagnostics of Wells syndrome should be carried out with skin granulomatous diseases and hypereosinophilic syndrome that may be characterized by similar clinical findings. Verification of Wells syndrome diagnosis is complicated due to its rareness, low awareness of dermatologists and pediatricians about this pathology, as well as ignoring the need to carry out histological tests during the disease exacerbation.
EXCHANGE OF EXPERIENCE
Psoriasis is one of the most debatable topics in modern dermatology among the wide range of other dermatological diseases in childhood. The article provides current data on the epidemiology of psoriasis, its clinical features in different age groups, its triggering factors, differential diagnostics, possible comorbid conditions, as well as the quality of life of patients and their relatives.
The graft-versus-host disease (GvHD) is frequent complication, it occurs in 50% of patients after allogeneic hematopoietic stem cell transplantation (HSCT) and it is one of the major causes of mortality not associated with disease recurrence. Skin lesion in the symptom complex of acute GvHD develops within the first 100 days after HSCT, and it is complicated diagnostic and therapeutic problem. Significant immunosuppressive status of children during the posttransplant period enhances and changes the course of dermatoses, infections, drug toxicity. Finally, it can lead to immunoallergic processes with possible development to generalized life-threatening diseases of the skin and mucous membranes. Meanwhile, toxic, allergic and infectious skin lesions can be present simultaneously or develop sequentially. The description of the clinical picture of skin lesions in acute GvHD is really crucial and has scientific and practical significance due to relatively small frequency of HSCT in children with oncology diseases. The article summarizes data on etiology, pathogenesis, clinical forms, diagnostic and treatment methods of cutaneous complications of the early post-transplantation period after HSCT.
Alopecia areata is one of the most common forms of hair loss in children. Meantime, its clinical picture is similar to trichotillomania, thus, it leads to incorrect diagnosis and management. The article provides major differential diagnosis signs for these related forms of alopecia and describes in details trichoscopic features of alopecia areata and trichotillomania.
Atopic dermatitis (AD) is one of the most common inflammatory diseases of childhood, and it is the first one in gradual development of allergic diseases, also known as «atopic march». Sensitization establishment during the AD uncontrolled course is associated with the high risk of developing of serious allergic pathologies, increase in the severity of the disease course, and patients’ quality of life reduction. Thereby, it is crucial to achieve quick jugulation of the inflammatory process in case of severe AD with early onset of disease. This article shows modern therapeutic approaches to disease control in children.
Atopic dermatitis (AD) is chronic recurrent inflammatory skin disease with a high prevalence and significant negative effect on patients' quality of life. AD pathogenesis is based on the complex interactions between genetic factors, immune mechanisms, state of the skin barrier and environmental effects. The climate itself is the complex of many components, such as: temperature, humidity, precipitation, wind and season. All of them play fundamental role in the natural ecosystem and human health establishment. The climate is changing rapidly, and these changes are progressing faster than ever in the last thousand years. This review shows how climate and environmental changes can affect the course of AD. The data on utilization efficiency of emollient plus for moderating of climatic conditions adverse effects on epidermal structures at patients with AD is presented.
A DOCTOR’S AID
Background. Skin mastocytosis is rare disease that is diagnosed in most children under the age of 2 years. The date on rash regression dynamics and disease symptoms is not fully presented in the literature.
Objective. The aim of the study was to analyze risk factors associated with clinical manifestations and regression time of skin mastocytosis in children.
Methods. The study includes data on 28 children aged from 3 months to 12 years who has undergone outpatient care and observation in Moscow Scientific and Research Center of Dermatovenerology and Cosmetology of Moscow City Health Department in the period between January 2016 and November 2019. The data about diagnosis was obtained from medical records.
Results. Maculopapular skin mastocytosis (MPSM) was diagnosed in 28.6% of children, solitary mastocytoma — in 71.4%. The analysis of clinical course of skin mastocytosis has shown constantly relapsing process and slow spontaneous rash regression in more than 50% of children. Diffuse skin rash, flushing reactions, persistent skin itching or its combination with hepatomegaly or neurological symptoms were prevalent among MPSM manifestations. Risk factors affecting delayed regression of skin mastocytosis in children with MPSM are: late onset, area of skin lesions, comorbidities, severity of reticular vascular pattern at dermatoscopy. Severity of skin lesions did not affect the tryptase activity. The major risk factor affecting the delayed regression of solitary mastocytoma is rash injury (OR 6.10, 95% CI 3.66–16.73). The severity of reticular vascular pattern in skin mastocytosis foci has varied depending on the severity of skin lesions.
Conclusion. Half of all children with skin forms of mastocytosis have delayed rash regression. This causes high concern among parents and violates social adaptation of children. Timely assessment of risk factors alongside with dynamic assessment of the dermatoscopy patterns and tryptase activity are important for implementation of correct follow-up monitoring and management for children. Rash (of any localization) injuries should be avoided to prevent delayed regression of the disease in children with skin forms of mastocytosis.
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.
Background. Malformations in epidermal barrier in children with atopic dermatitis (AD) can cause transcutaneous sensitization with further development of allergic diseases that can worsen the AD course and significantly reduces patients’ quality of life.
Objective. The aim of the study was to determine the effect of topical treatment and maintenance therapy with pimecrolimus 1% cream (PIM) and topical glucocorticosteroids (tGCS) in infants with AD on reducing the risk of developing transcutaneous sensitization (due to the levels of specific IgE to the cow milk protein over time) and on reducing the disease severity (by the EASI scale).
Methods. The study included children aged from 1 to 4 months with early manifestations of moderate and severe AD. The severity of AD was estimated via the EASI scale at start of observation, then at 6, 9 and 12 months of life. The class and level of specific IgE to cow milk proteins (CMP) were determined by the ImmunoCAP method at the point of enrolment and at the ages of 6 and 12 months. Statistical analysis of studied indicators dynamics and their comparison in research groups was carried out using multifactorial dispersion analysis.
Results. The study included 36 patients. All patients have received standard tGCS therapy in combination with emollients (wet wrap) for 10 days. The maintenance therapy was prescribed in postacute period. It included topical calcineurin inhibitor PIM 2 times/day for 3 months, then double application (morning/evening) 3 times/week up to the age of 1 year old (group 1). Other group had maintenance therapy — tGCS2 times/week for 3 months, and then at AD aggravation (group 2). Group 1 has shown lower level of sensitization to CMP at the age of 6 and 12 months and more significant decrease in AD severity according to EASI scale compared to group 2.
Conclusion. The treatment with PIM is effective in therapy of AD and prevention of transcutaneous sensitization in infants.
ISSN 1682-5535 (Online)