CLINICAL AND MORPHOLOGICAL SKIN LESIONS IN THE STRUCTURE OF CONNECTIVE TISSUE DYSPLASIA

Abstract

Relevance of the problem. Connective tissue dysplasia (CTD) is one of the most important medical and social problems of congenital pathology due to its progressive course, multiorgan involvement, and often unfavorable outcome. Data on the prevalence of CTD are contradictory: the detection rate of individual manifestations in the population varies from 8.5% to 85.4%, which is due to different classification and diagnostic approaches. Currently, differentiated and undifferentiated connective tissue dysplasia (UCTD) are distinguished. UCTD is diagnosed when a patient's set of phenotypic features does not fit the clinical picture of any known differentiated hereditary disease . The literature contains numerous publications on cardiovascular and musculoskeletal system damage in UCTD. According to the authors, skin pathology in UCTD reaches 18.0%. However, the description and frequency of manifestations of individual signs of skin pathology in different studies are contradictory, and their diagnostic significance has not been determined. Few studies by dermatologists have been devoted to assessing the impact of the dysplastic process on the course of associated skin pathology. A direct correlation has been established between the severity of clinical manifestations of atopic dermatitis and the severity of CTD. Researchers point to the pathogenetic significance of a deficiency of copper, zinc, and magnesium ions in the development of the main clinical symptoms of CTD [82]. It has been established that a deficiency of these micro- and macroelements leads to increased degradation of collagen and elastin fibers, as well as hyaluronan polysaccharide threads , which ultimately leads to a decrease in the mechanical strength of connective tissue. However, the diagnostic significance of determining the level of micro- and macroelements in patients with CTD, its relationship between external and internal signs, and minor developmental anomalies (MDAs) has not been sufficiently studied. Thus, the study of the signs and MARs of the skin in UCTD, the assessment of the frequency of occurrence and the determination of their diagnostic significance taking into account the principles of medical statistics, the development of a diagnostic algorithm taking into account modern regulatory documents, with the aim of providing highly qualified specialized dermatovenereological medical care that ensures the appropriate quality of preventive measures aimed at the early detection of UCTD using clinical and laboratory data, is relevant and is of scientific and practical interest.