Vestnik dermatologii i venerologii最新文献

{"title":"Junctional epidermolysis bullosa: genotype-phenotype correlations","authors":"A. Kubanov, V. Chikin, A. Karamova, E. Monchakovskaya","doi":"10.25208/vdv1391","DOIUrl":"https://doi.org/10.25208/vdv1391","url":null,"abstract":"Junctional epidermolysis bullosa most commonly results from mutations in theLAMA3, LAMB3, LAMC2, COL17A1, ITGA6 and ITGB4genes. Junctional epidermolysis bullosa is characterized by clinical heterogeneity. To date, scientific findings allow to evaluate correlations between the severity of clinical manifestations and genetic defects underlying in the development of the disease. A systematic literature search was performed using PubMed and RSCI, and keywords including junctional epidermolysis bullosa, laminin 332, collagen XVII, 64 integrin. The review includes description of clinical findings of junctional epidermolysis bullosa, mutation location and types, its impact on protein production and functions. To evaluate the impact of gene mutation on protein functions, this review explores the structure and functions of lamina lucida components, including laminin 332, collagen XVII and 64 integrin, which are frequently associated with the development of junctional epidermolysis bullosa. The correlation between severe types of junctional epidermolysis bullosa and mutations resulting in premature stop codon generation and complete absence of protein expression has been described. Although, genotype-phenotype correlations should be analyzed carefully due to mechanisms which enable to improve protein expression.","PeriodicalId":23618,"journal":{"name":"Vestnik dermatologii i venerologii","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-11-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"43243062","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Performance results of medical organizations providing medical care in the field of dermatovenereology in 2019-2021 in the Russian Federation","authors":"E. Bogdanova, A. Kubanov","doi":"10.25208/vdv1337","DOIUrl":"https://doi.org/10.25208/vdv1337","url":null,"abstract":"The article presents the results of analysis of resources and performance rates of medical organizations providing medical care in the field of dermatovenereology over 2019-2021 in the Russian Federation. The changes in the rates of provision of the population of the Russian Federation with doctors - dermatovenereologists are reflected. The rates of provision of the population of the Russian Federation with round-the-clock and daytime dermatovenerological beds are presented. The rates of dermatovenereologic medical care provided to the population of the Russian Federation in 2021 in outpatient, inpatient and day hospital conditions were compared with those before the pandemic. The dynamics of incidence of sexually transmitted infections as well as dynamics of prevalence and incidence of skin and subcutaneous tissue disorders among the population of the Russian Federation, and specifically in different age groups, during the period under review is presented.","PeriodicalId":23618,"journal":{"name":"Vestnik dermatologii i venerologii","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-11-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"44563806","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"MECHANISMS OF ROSACEA PATHOGENESIS. A PHENOTYPIC APPROACH TO THERAPEUTIC TACTICS. DEMONSTRATION OF A CLINICAL CASE","authors":"M. Drozhdina, V. Bobro","doi":"10.25208/vdv1310","DOIUrl":"https://doi.org/10.25208/vdv1310","url":null,"abstract":"Rosacea is a widespread disease among the population, infected 5.5%. It is known that the activation of the innate and susceptible immune system, as well as neurovascular dysregulation underlying the manifestation of rosacea signs, do not fully explain all the clinical peculiarities of this disease. This dictates the prospect of continuing the diagnostic search and the therapeutic approach with the personalization of management tactics individually for the patient, depending on the prevalence of the manifestation of the characteristics of the course of the disease. A genetic predisposition to modified immune reactivity suggests an association of rosacea with single nucleotide polymorphisms in genes associated with the major histocompatibility complex. Currently, rosacea is considered as a single disease with several main phenotypes: facial erythema, telangiectasias, papulo-pustular manifestations, phimosis and ophthalmic rosacea. Most experts tend to think about the phenotypic approach in modern rosacea therapy, which should be aimed at achieving an improvement in the patient's overall well-being, focusing on those aspects that are most disturbing to the patient, based on the principles of evidence-based medicine. A clinical case of a patient with a papulo-pustular rosacea phenotype is presented. The high effectiveness of the therapy with systemic isotretinoin and topical support in the form of metronidazole and tacrolimus was demonstrated.","PeriodicalId":23618,"journal":{"name":"Vestnik dermatologii i venerologii","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"42650063","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A clinical case of the debut of pemphigus foliaceus against the background of vaccination against coronavirus infection (COVID-19)","authors":"V. Vlasova, Martynov Martynov, Svischenko Svischenko","doi":"10.25208/vdv1336","DOIUrl":"https://doi.org/10.25208/vdv1336","url":null,"abstract":"The article describes the onset of pemphigus foliaceus against the background of vaccination with the combined vector vaccine \"Gam-COVID-Vac\" (\"Sputnik V\"). A feature of this clinical case is the duration of the period from the moment of development of the first symptoms of the disease to clinical and immunohistochemical confirmation of the diagnosis, which, in fact, indicates a low availability of specialized medical care for rare dermatoses. In particular, the waiting period for taking material for histological examination lasted for several months, which, in fact, is not an isolated case. At the same time, against the background of an increase in clinical symptoms, this became an additional factor in the patient's self-referral to a specialized federal state medical institution, where, within two weeks, the diagnosis was confirmed by immunohistochemical analysis methods and inpatient treatment was carried out with a pronounced positive effect. In addition, the analysis of the medical care provided to the patient showed insufficient alertness and the level of training of dermatovenereologists in diagnosing rare dermatoses accompanied by blistering rashes. In particular, the patient initially underwent several courses of antifungal therapy in combination with intravenous infusion of systemic glucocorticosteroids. The article also presents an analysis of the frequency of occurrence of this disease in different countries for more than 100 years.","PeriodicalId":23618,"journal":{"name":"Vestnik dermatologii i venerologii","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"47514090","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"CURRENT STATE ON THE SYNDROM OF RED SCROTUM","authors":"N. Abuduyev, M. Vasiliev, Xenia Plahova, G. Katunin","doi":"10.25208/vdv1357","DOIUrl":"https://doi.org/10.25208/vdv1357","url":null,"abstract":"Red scrotum syndrome is a chronic, difficult-to-recognize dermatosis affecting middle-aged and elderly people. It is manifested by erythema of the scrotum with clear boundaries, without neck. The etiology and pathogenesis of the disease have not been established. Hypotheses about the causes of dermatosis as a consequence of uncontrolled use of topical steroids, neurological inflammation, formation of microbial biofilm are based on anamnestic data and the results of trial treatments. Etiologically and pathogenetically justified therapy has not been proposed. Therapy with the use of doxycycline, gabapentin, pregabalin, indomethacin, tacrolimus was carried out in small groups of patients. The lack of clarity in the etiology and understanding of the mechanism of development of the disease explains the lack of a unified focus of the proposed therapies based on isolated clinical cases. In general, the problem of diagnosis and treatment rests on the absence of major clinical, epidemiological and laboratory studies that allowed to establish the pathophysiology and assess the true prevalence of red scrotum syndrome.","PeriodicalId":23618,"journal":{"name":"Vestnik dermatologii i venerologii","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49160256","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Rare case of cutaneous collagenous vasculopathyin a patient with chronic HCV infection 1b subtype","authors":"Rada Shpiluyk, I. Belousova, D. Sulima, Safura Sulejmanova, Y. Gorbunov, Natalia Blum","doi":"10.25208/vdv1343","DOIUrl":"https://doi.org/10.25208/vdv1343","url":null,"abstract":"The article is devoted to a rare disease of cutaneous collagenous vasculopathy (CCV), which is characterized by common leather telangiectasias and specific histological signs: extended capillaries in the surface layers of the dermis, the walls of which are thickened due to hyaline deposits containing type IV collagen. According to literature, there are no publications about CCV in Russian sources. In foreign literature, only 60 cases are described, the first of which is mentioned in 2000. The patient turned to the EXCLUSIVE medical clinic to resolve the issue of the tactics of treatment of chronic HCV-infection 1b subtype and complaints of common telangiectasia on the skin and mucous membrane of both sclera, without subjective sensations. The first rashes appeared in 2008 and gradually progressed. When performing histological examination, expanded capillaries were found in the surface layers of the dermis, the walls of which are thickened due to protein deposits containing type IV collagen. The described case of CCV is the first in Russian literature and shows the need for histological and immunohistologycal studies to establish a final diagnosis.","PeriodicalId":23618,"journal":{"name":"Vestnik dermatologii i venerologii","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"42418408","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Lentigo melanoma associated with Laser-Trel syndrome","authors":"V. Smolyannikova, A. Alexandrova, Andrey Vladimirovich Filatov","doi":"10.25208/vdv1339","DOIUrl":"https://doi.org/10.25208/vdv1339","url":null,"abstract":"Laser-Trela syndrome is characterized by the sudden appearance of a large amount of seborrheic keratosis . The syndrome is associated with the development of malignant tumors of internal organs. The article presents for the first time the observation of 82-year-old patient with lentigo maligna melanoma, who had multiple seborrheic keratosis in a short time. The observation is interesting for the atypical clinical picture of seborrheic keratosis, and the association with lentigo melanoma.","PeriodicalId":23618,"journal":{"name":"Vestnik dermatologii i venerologii","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"42818705","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Role of innate immune system cells in psoriasis","authors":"E. Sorokina, I. Bisheva","doi":"10.25208/vdv1330","DOIUrl":"https://doi.org/10.25208/vdv1330","url":null,"abstract":"Psoriasis is an immune-mediated disease with a complex pathogenesis. The close relationship between the development of psoriasis and the adaptive immune response is already known. However, recent data have shown that innate immune cells also play an important role in the development of psoriasis. Congenital lymphoid cells, dendritic cells, T cells, NK cells, and NKT lymphocytes are activated in psoriasis, contributing to disease pathology through IL-17-dependent and independent mechanisms. During disease progression, T cells secrete proinflammatory cytokines that induce and exacerbate the course of psoriasis. T cells have memory cell properties that respond rapidly to secondary stimulation, which contributes to disease relapse. This article presents an overview of recent findings demonstrating the role of innate immunity in psoriasis.","PeriodicalId":23618,"journal":{"name":"Vestnik dermatologii i venerologii","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"44456895","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Psoriatic erythroderma: comparative patient profile and association with psoriatic arthritis","authors":"E. Bogdanova","doi":"10.25208/vdv1328","DOIUrl":"https://doi.org/10.25208/vdv1328","url":null,"abstract":"Background: Psoriatic erythroderma is rare and the most severe form of psoriasis characterized by involvement of 80-90% of body surface area. \u0000Aims: to compare clinical and demographic characteristics of patients with psoriasis, depending on presence of psoriatic erythroderma in medical history; to assess the association between psoriatic arthritis and a history of psoriatic erythroderma. \u0000Materials and methods: patients aged 18 years and older with a duration of psoriasis of 10 years, from the psoriasis patients registry were included. \u0000Comparison of continuous quantitative variables was performed using t-test or Mann-Whitney U-test. The 2 test or Fisher's exact test was used to compare qualitative binary variables. \u0000The odds ratio of psoriatic arthritis in patients with and without a history of psoriatic erythroderma was calculated, unadjusted and adjusted for other independent variables. \u0000Results: The number of patients included in the analysis was 1,870. A history of psoriatic erythroderma (PE) had 227 (12.1%) of them. There were no significant differences in patient age at inclusion to the registry (47.113.1 years vs. 47.713.3 years), and prevalence of PE among men and women (12.6% vs. 11. 4%). \u0000Higher prevalence of arterial hypertension (47.9% vs 32.7%), angina pectoris (6.4% vs. 1.9%), myocardial infarction (3. 2% vs. 1.0%), biliary dyskynesia (14.3% vs. 3.9%), fatty liver (12.2% vs. 7.7%), cholelithiasis (8. 6% vs. 3.6%), and toxic liver disease (2.2% vs. 0.5%) among patients with vs without history of PE was found. \u0000Psoriatic arthritis was diagnosed in 67.0% patients with a history of psoriatic erythroderma. The odds of having psoriatic arthritis in patients with a history of psoriatic erythroderma were 4 times higher than in patients without erythroderma: 4.043 [2.975-5.493]. When adjusted for sex, age, duration of psoriasis, and body mass index, the odds ratio and statistical significance were maintained: 4.064 [2.939-5.619]. \u0000Conclusions: compared to patients without PE those with a history of psoriatic erythroderma have a greater comorbidity burden: a higher prevalence of cardiovascular diseases, diseases of the liver and biliary tract. A significant association between psoriatic arthritis and a history of PE was established.","PeriodicalId":23618,"journal":{"name":"Vestnik dermatologii i venerologii","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-10-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"48715148","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A patient with connective tissue dysplasia at a cosmetologist's appointment: features of diagnosis and management","authors":"O. B. Borzykh","doi":"10.25208/vdv1355","DOIUrl":"https://doi.org/10.25208/vdv1355","url":null,"abstract":"Patients with various manifestations of involutional skin changes often come to an appointment with a cosmetologist and a dermatologist. Among such patients, a group can be distinguished who have premature aging, or rather more pronounced signs of facial aging, in comparison with their peers. And it is among this group of patients that patients with connective tissue dysplasia are often found. \u0000In this clinical case, an example of a patient with connective tissue dysplasia, with multiple organ disorders, with skin manifestations of dysplasia is presented. And on her example, the tactics of managing such patients, diagnostic features and possible errors in the management of patients with connective tissue dysplasia are analyzed. Particular attention should be paid to the joint management of such patients with doctors of other specialties, respectively, the organ systems involved and recommendations for patients with connective tissue dysplasia regarding lifestyle.","PeriodicalId":23618,"journal":{"name":"Vestnik dermatologii i venerologii","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-10-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"43936777","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}