Vol 98, No 6 (2022)

REVIEWS

Junctional epidermolysis bullosa: genotype-phenotype correlations

Kubanov A.A., Chikin V.V., Karamova A.E., Monchakovskaya E.S.

Abstract

Junctional epidermolysis bullosa most commonly results from mutations in the LAMA3, LAMB3, LAMC2, COL17A1, ITGA6 and ITGB4 genes. 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”, “α6β4 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 α6β4 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.

Vestnik dermatologii i venerologii. 2022;98(6):17-38
pages 17-38 views

Molecular markers of recurrence of basal cell skin cancer

Saytburkhanov R.R., Kubanov A.A., Kondrakhina I.N., Plakhova X.I.

Abstract

Basal cell carcinoma is the most common malignant neoplasm, andits incidence has been rapidly increasing worldwide. This malignancy is a serious public health problem that can lead to disability and severe aesthetic damage.

The results of the analysis of gene expression and proteomic profiling of tumor cells and the tumor microenvironment indicate that certain molecules involved in the pathogenetic pathways of basal cell skin cancer may represent new prognostic biomarkers or serve as the basis for their development.

The purpose of this review is to summarize information on known genes, proteins, and enzymes that can be used to diagnose, monitor therapy, and predict the course of basal cell skin cancer.

The PubMed, MedLine, Web of Science and RSCI databases were used to search for the necessary literature.

Vestnik dermatologii i venerologii. 2022;98(6):39-47
pages 39-47 views

Revisiting the question of assessment by severity and classification of acne

Araviiskaia E.R., Samtsov A.V., Sokolovskiy E.V., Bakulev A.L., Murashkin N.N., Karamova A.E.

Abstract

The paper presents an analysis of different variants of acne classifications and the assessment of acne severity, carried out by the working group of the RODVK Committee for Classifications in Dermatovenereology. There is a division of acne vulgaris according to age and severity. Assessing the severity of acne is important for choosing the correct prescriptions in daily practice and for the evaluation of treatment outcomes. Most of existing acne grading systems are based either on a global grading of severity or on calculating the number of lesions in a variety of anatomic locations. For clinical practice of dermatovenereologists, as well as for educational and scientific purposes, the working group proposed to supplement the acne classification based on severity grading with counting the number of the lesions, and to take into account localization, rash prevalence and scarring when determining the acne severity.

Vestnik dermatologii i venerologii. 2022;98(6):48-54
pages 48-54 views

Phototherapy combined with systemic agents for mycosis fungoides

Vorontsova A.A., Karamova A.E.

Abstract

The choice of tactics for managing a patient with mycosis fungoides is determined by the stage of the disease. In the early stages of mycosis fungoides, a treatment approach using external therapy and various ultraviolet irradiation spectra (UVB-311 nm and PUVA therapy) is preferable. With insufficient efficiency and / or short remissions in some patients, it is possible to use combined methods in the early stages: PUVA therapy or UVB-311 nm therapy. in combination with systemic therapy, including interferon (IFN) α2b preparations, methotrexate and retinoids. The results of original studies demonstrate an increase in the overall response rate when combining PUVA therapy with IFN-α or retinoids in patients with stages IB-IIB. The effectiveness of combined treatment regimens using UVB-311 nm remains insufficiently studied.

Vestnik dermatologii i venerologii. 2022;98(6):55-64
pages 55-64 views

ORIGINAL STUDIES

The role of proteins implicated in skin steroidogenesis in acne vulgaris development

Kirichenko A.K., Bardetskaya Y.V., Fefelova Y.A., Kotova K.V., Tokmakova V.O., Ruksha T.G.

Abstract

Background. Acne vulgaris belongs to androgen-dependent diseases where androgens cause sebaceous gland hypertrophy with a subsequent enlargement of sebum production.

Aims. Determination of the expression profile of steroidogenesis-related proteins in normal skin and skin of patients with acne.

Material and methods. Immunohistochemical assay was carried out to characterize TSPO, StAR, CYP11A1, and 3â-hydroxysteroid dehydrogenase expression in skin of patients with acne vulgaris and in normal skin.

Results. Expression levels of a protein TSPO which is involved in a steroidogenesis-limited step was increased in mature sebocytes of acne vulgaris patients. StAR, CYP11A1, and 3â-hydroxysteroid dehydrogenase immunovisualization was found both in normal skin and in the skin of acne patients.

Conclusions. The revealed alterations in expression of steroidogenesis-related protein in acne skin may indicate not only the conversion of steroids produced by external steroidogenesis organs but elevated steroids local production as well. The fact may account both for sebaceous gland hyperplasia and inflammation sustenance in skin.

Vestnik dermatologii i venerologii. 2022;98(6):65-72
pages 65-72 views

Psoriatic erythroderma: comparative patient profile and association with psoriatic arthritis

Bogdanova E.V.

Abstract

Background. Psoriatic erythroderma is rare and one of the most severe forms of psoriasis characterized by involvement of 80–90% of body surface area.

Aims. 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.

Materials and methods. Patients aged 18 years and older with a duration of psoriasis of >10 years, from the psoriasis patients registry were included.

Comparison of continuous quantitative variables was performed using t-test or Mann–Whitney U-test. The χ2 test or Fisher's exact test was used to compare qualitative binary variables.

The odds ratio of psoriatic arthritis in patients with and without a history of psoriatic erythroderma was calculated, unadjusted and adjusted for other independent variables.

Results. 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 (47.1 ± 13.1 years vs. 47.7 ± 13.3 years), and prevalence of PE among men and women (12.6% vs. 11. 4%).

Higher 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.

Psoriatic 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].

Conclusions. 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.

Vestnik dermatologii i venerologii. 2022;98(6):73-80
pages 73-80 views

Comparative analysis of sexual behavior and assessment of awareness of STIs and HIV infection among young people of two generations

Khryanin A.A., Shpiks T.A., Russkikh M.V.

Abstract

Background. Over the past 20 years, various preventive measures have been taken to educate the young generation, which is the most vulnerable to sexually transmitted infections (STIs) and HIV infection. A comparative analysis of the results of a survey of young people (belonging to two different generations: “millennials” and “zoomers”) on sexual behavior and awareness of STIs and HIV infection allows us to assess the existing characteristics of the sexual behavior of young people and contribute to the further improvement of preventive measures among young people regarding STI/HIV infection.

Aims. Comparative analysis of sexual behavior and assessment of the level of knowledge about STIs and HIV infection among young people (generations Y and Z).

Materials and methods. For a comparative assessment of the level of awareness on the issues of prevention and risky sexual behavior in relation to STIs and HIV infection, data from earlier sociological surveys of young people in 2001–2002 and in 2017–2020 in the city of Novosibirsk were used. Among the respondents, a questionnaire was used, consisting of 23 questions, developed by the staff of the Department of Dermatovenereology of the First St. Petersburg State Medical University named after Academician I. P. Pavlov and the Center for the Sociology of Deviance and Social Control of the Institute of Sociology of the Russian Academy of Sciences, adapted by the authors. The questionnaire consisted of two parts, the first part contained questions characterizing students' knowledge about the ways of transmission of STI/HIV infection and methods of prevention. The second part of the questionnaire is devoted to the issues of risky sexual behavior of students. Statistical analysis was carried out using Microsoft Excel and SPSS programs. The χ2 test and the independent Student's t-test were evaluated. Statistical significance was set as p < 0.05.

Results. A total of 772 young men took part in the study, of which 321 students were interviewed in 2001–2002 (Generation Y), and 451 students in 2017–2020 (Generation Z). The results show that generations of young people (“milleneals” and “zoomers”) differ from each other in terms of the source of information about STIs, sexual intercourse with strangers, condom use, and the frequency of same-sex sexual intercourse.

Conclusions. There is a positive trend in the sexual behavior of the younger generation. More structured promotion of STI/HIV prevention is needed, which will take into account current trends and features of obtaining information from young people.

Vestnik dermatologii i venerologii. 2022;98(6):81-88
pages 81-88 views

The use of 585 and 1064 nm laser for the treatment of basal cell skin cancer

Saytburkhanov R.R., Kondrakhina I.N., Plakhova X.I., Kubanov A.A.

Abstract

Background. Basal cell skin cancer is the most common malignant epithelial skin neoplasm in fair-skinned people and accounts for two-thirds of all skin cancers in this population. Tumor lesions have a supporting vascular network that serves as a target for vascular selective lasers.

Aims. To assess the effects of laser radiation at a wavelength of 585 and 1064 nm on the vasculature of various forms of basal cell skin cancer using dermatoscopy and in vivo confocal microscopy.

Materials and methods. The study included 20 patients with 20 lesions of histologically confirmed basal cell skin cancer treated with a pulsed dye laser (wavelength 585 nm) and a long-pulse neodymium laser (wavelength 1064 nm) in the conditions of the consultative and diagnostic center of the Federal State Budgetary Institution “GNTSDK” of the Ministry of Health RF in the period from 2021 to 2022. The effectiveness of therapy was assessed using non-invasive methods for examining the skin.

Results. According to the results of the two-year follow-up period, 100% effectiveness of therapy in both groups is noted, in 2 patients treated with a neodymium laser, the formation of hypertrophic scars in the treatment area is noted.

Conclusions. Treatment of patients with basal cell skin cancer with selective vascular lasers may be an effective treatment option. Additional studies with a larger sample of observations and a long monitoring period are needed to confirm the effectiveness of this treatment method.

Vestnik dermatologii i venerologii. 2022;98(6):89-100
pages 89-100 views

GUIDELINES FOR PRACTITIONERS

About the relationship of rosacea-like dermatitis

Bakulev A.L., Bakuleva M.V.

Abstract

The literature review is devoted to the problem of rosacea-like dermatitis developing in patients with rosacea against the background of irrational uncontrolled use of topical glucocorticosteroids or cosmetics. The clinical picture of the combined pathology consists of symptoms characteristic of both diseases, which makes diagnosis difficult.

Management tactics of patients with combined pathology, first of all, involves the elimination of symptoms of rosacea–like dermatitis, dominating the clinical picture, using metronidazole, calcineurin inhibitors, azelaic acid, in severe cases — low doses of systemic isotretinoin. The possibility of using activated zinc pyrithione, which has antimicrobial, anti-fungal and anti-inflammatory effects, as a topical remedy to eliminate the symptoms of rosacea-like dermatitis is discussed.

Vestnik dermatologii i venerologii. 2022;98(6):101-106
pages 101-106 views

CLINICAL CASE REPORTS

A patient with connective tissue dysplasia at a cosmetologist's appointment: features of diagnosis and management

Borzykh O.B.

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. Сonnective tissue dysplasia are often found in this group of patients.

An example of a patient with connective tissue dysplasia, with multiple organ disorders, with skin manifestations of dysplasia is presented. 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.

Vestnik dermatologii i venerologii. 2022;98(6):107-112
pages 107-112 views


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