Abstract
The group of scleroderma diseases includes a number of clinical entities, the main symptom of which is skin tightening. Scleroderma is a prominent example of these diseases, characterized by excessive synthesis and deposition of collagen in organs and tissues. A patient with juvenile systemic scleroderma with induration of the skin and underlying tissues, and persistent contractures of large joints since childhood, is described. This clinical example illustrates disease course peculiarities and differential diagnosis of systemic and limited (focal) scleroderma and scleroderma-like conditions in pediatric patients. The feasibility of pathogenetic therapy aimed at improving patient's the quality of life with formed disease phenotype is shown.
Highlights
The group of scleroderma diseases includes a number of clinical entities, the main symptom of which is skin tightening
Scleroderma is a prominent example of these diseases, characterized by excessive synthesis and deposition of collagen in organs and tissues
The feasibility of pathogenetic therapy aimed at improving patient's the quality of life with formed disease phenotype is shown
Summary
A patient with juvenile systemic scleroderma with induration of the skin and underlying tissues, and persistent contractures of large joints since childhood, is described. This clinical example illustrates disease course peculiarities and differential diagnosis of systemic and limited (focal) scleroderma and scleroderma-like conditions in pediatric patients. Склеродермия является ярким представителем этих заболеваний, характеризующихся избыточным синтезом и отложением коллагена в органах и тканях. Системная склеродермия (ССД), или прогрессирующий системный склероз, – полиорганное заболевание, в основе которого лежат иммунные нарушения и вазоспастические сосудистые реакции по типу феномена Рейно, сопровождающиеся активацией фиброзообразования и избыточным отложением компонентов внеклеточного матрикса (коллагена) в тканях и внутренних органах [1].
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