CLINICAL CASE: RARE COURSE OF JUVENILE SCLERODERMA IN RESIDENTS OF DONETSK REGION

Abstract

The article presents a clinical case of a rare onset and a special clinical course of juvenile scleroderma. A clinical case of a child who was born and lives in the ecologically unfavorable industrial Donetsk region is described. The literature review of influence of unfavorable environment on morbidity and features of autoimmune pathology course in patients of Donetsk region is offered. In particular, the literature indicates that negative environmental factors lead to an increase in the progression of systemic scleroderma; over the past 20 years, there has been a tendency to increased mortality of children suffering from it, which is associated with deteriorating environmental conditions in industrial regions. The growing incidence and prevalence of scleroderma, the variety of clinical manifestations, and difficulties in early diagnosis of the disease make it important to study the options for the course of this pathology in children and adolescents in the early stages of the disease. A feature of this clinical case was the onset of juvenile scleroderma with severe convulsive syndrome at the age of 8 years, which required Finlepsin at a dose of 200 mg daily. Further course was also uncharacteristic: within 2 years, there was a linear indentation in the forehead on the right side; the patient was diagnosed with linear "saber-shaped" limited scleroderma, Parry–Romberg facial hemiatrophy syndrome, and further developed manifestations of systemic involvement, lesions of internal organs: pneumofibrosis and scleroderma esophagitis. The management and observation of the patient are presented here. It was emphasized that early aggressive intervention led to the prevention of severe organ pathology and death. The described clinical case expands the knowledge of physicians on the clinical polymorphism of the onset and course of the disease, which allows faster and more accurate identification of the disease, timely and adequate therapy, and will lead to earlier stabilization and remission of the disease.

Keywords

• systemic scleroderma, children, clinic, diagnosis, treatment