Vol 101, No 4 (2025)

Pemphigus is a group of autoimmune bullous dermatoses that affect the skin and/or mucous membranes, potentially fatal outcome. The most common clinical form is pemphigus vulgaris (PV) which is characterized by IgG circulating in the blood and fixed in the epidermis directed against desmoglein-3 (Dsg3) in mucosal lesions and against desmoglein-1 (Dsg1) in skin lesions. The extracellular part of Dsg3 consists of five domains (EC1–EC5) which ensure strong adhesion between adjacent molecules of neighboring cells. The important role of Dsg3 and its individual extracellular domains, primarily EC1 and EC2, in initiating the autoimmune process and generating the clinical phenotype of the disease is described. Variability of antibody pathogenicity depending on its domain specificity may be used as a marker for assessing the prognosis of the disease, and it also opens up new opportunities for developing targeted therapeutic strategies.

Epidermolysis bullosa (EB) is a phenotypically and genetically heterogeneous group of hereditary dermatoses characterized by the formation of blisters on the skin and/or mucous membranes with minimal mechanical exposure. The study of the pathogenesis and development of therapeutic strategies for EB present significant challenges. In this regard, experimental animal models of EB, especially using laboratory mice, are important in modern science. Genetically modified lines reproducing key mutations in the corresponding genes (Krt5, Krt14, Plec, Lama3, Lamb3, Lamc2, Col7a1, etc.) successfully mimic phenotypic manifestations characteristic of human forms of EB and allow us to study the stages of the pathological process development, as well as to study the molecular basis of the disease and initiate the development of new effective methods of treatment. The advent of genome editing using CRISPR-Cas9, which allows targeted mutations in genes of interest, has simplified the disease modeling process. Further improvement of models is necessary for effective translation of experimental data into clinical practice.

Background. Psoriasis is a common chronic skin disease with frequent pathological musculoskeletal changes which has a significant impact on health and has a negative socioeconomic effect. Data on disease control and treatment strategies in routine clinical practice in Russia are limited.

Aim. To describe the severity of the disease, response to therapy, and quality of life in patients who received continuous systemic therapy for ≥ 24 weeks for moderate to severe psoriasis.

Methods. In the Russian Federation, CRYSTAL retrospective observational crossover study enrolled adult (18–75 years old) patients (n = 100) with moderate to severe psoriasis who received continuous systemic treatment for ≥ 24 weeks.

Results. For most patients (99.0%), their current treatment was monotherapy with a genetically engineered biological agent (GEBA) (96.0% of patients) or other systemic drugs (3.0% of patients); 1 patient received combination therapy. The average absolute PASI score was 3.4 ± 4.0 and 6.7 ± 9.6 in GEBA group and the group receiving other systemic drugs, respectively. Absolute PASI score of > 1, > 3, and > 5 was observed in 55.2%, 39.6%, and 28.1% of patients receiving GEBA, and in 66.7%, 66.7%, and 33.3% of patients receiving other systemic drugs. The average overall DLQI score was 2.6 ± 4.2.

Conclusion. A significant number of patients had low absolute PASI scores after continuous treatment for at least 24 weeks and good satisfaction with the therapy. However, only 21.0% of patients achieved regression of psoriatic lesions, and more than one-third of patients reported that the disease had an impact on their quality of life, which still indicated the need to improve treatment strategies in Russia to optimize outcomes in patients with moderate to severe psoriasis.

Background. Dermatoscopy has become a typical practice in providing assistance in dermatovenereology, cosmetology and oncological diseases. However, the low level of equipment of medical organizations with dermatoscopes remains. At the same time, the level of knowledge and skills of doctors in matters of dermatoscopy, the quality of relevant educational programs have not been analyzed before.

Aims. Conduct an analysis of the ongoing advanced training programs on dermatoscopy and assess the degree of implementation of dermatoscopy in the activities of medical organizations.

Methods. The available advanced training programs were analyzed and an anonymous survey of 402 doctors was conducted using a specially developed sociological questionnaire.

Results. Despite the variety of options and forms of training, the quality of training remains low, especially for correspondence educational programs. 66.7% and 45.5% of respondents, respectively, received training in dermatoscopy and dermato-oncology. At the same time, about 20% of specialists mastered programs of less than 6 hours. Almost 75% of respondents noted insufficient knowledge for full-fledged work with a dermatoscope (in most cases, this concerns the diagnosis of malignant neoplasms). Almost half of the respondents perform photo fixation during dermatoscopy, but most organizations do not provide for the transfer and storage of images.

Conclusion. It is advisable to create a methodological center on dermatoscopy issues based on the Department of Dermatovenereology and Cosmetology of the Russian Medical Academy of Postgraduate Education. It is necessary to develop and implement a standard sample program on dermatoscopy issues in the amount of at least 36 hours. It is necessary to develop a unified protocol for a dermatoscopy report, both a primary dermatoscopy report and a dynamic control protocol. The terminology used in dermatoscopy in Russian requires separate analysis.

Diffuse hair loss refers to non-scarring hair loss caused by various reasons. Despite the variety of etiological factors that can cause hair loss, these types of alopecies share a common characteristic: cessation of hair growth during the anagen phase. The variety of underlying causes of hair loss in these forms of alopecia complicates both therapy choice and clinical management. In this article the authors share their own clinical experience in treating such patients. Modern drugs and topical agents allow for maximum results with the absence of side effects. Treatment efficacy was assessed by clinical tests (visual examination and pull test assessment), as well as objective diagnostic methods, including trichoscopy using TrichoSciencePro software, trichogram and phototrichogram. Two clinical cases with treatment of patients with telogen effluvium are presented.