mrjСовременная ревматологияModern Rheumatology Journal1996-70122310-158XIMA-PRESS, LLC10.14412/1996-7012-2021-2-64-68mrj-1124Research ArticleКЛИНИЧЕСКИЕ НАБЛЮДЕНИЯCLINICAL OBSERVATIONSФиброзирующая артропатия при ювенильной склеродермииFibrosing arthropathy in juvenile sclerodermahttps://orcid.org/0000-0003-3183-0464ДибровД. А.DibrovD. A.

Данил Алексеевич Дибров

115522, Москва, Каширское шоссе, 34А

Danil Alekseevich Dibrov

34A, Kashirskoe Shosse, Moscow 115522

dibrovd995@gmail.comhttps://orcid.org/0000-0002-1004-9647СтаровойтоваМ. Н.StarovoitovaM. N.

115522, Москва, Каширское шоссе, 34А

34A, Kashirskoe Shosse, Moscow 115522

https://orcid.org/0000-0002-0283-9681ДесиноваО. В.DesinovaO. V.

115522, Москва, Каширское шоссе, 34А

34A, Kashirskoe Shosse, Moscow 115522

ФГБНУ «Научно-исследовательский институт ревматологии им. В.А. Насоновой»Russian FederationФГБНУ «Научно-исследовательский институт ревматологии им. В.А. Насоновой»РоссияV.A. Nasonova Research Institute of RheumatologyRussian Federation2021230420211526468Copyright © Дибров Д.А., Старовойтова М.Н., Десинова О.В., 20212021Дибров Д.А., Старовойтова М.Н., Десинова О.В.Dibrov D.A., Starovoitova M.N., Desinova O.V.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://mrj.ima-press.net/mrj/article/view/1124

К группе склеродермических болезней относится ряд нозологий, основным признаком которых является уплотнение кожи. Склеродермия – яркий представитель этих заболеваний, характеризующихся избыточным синтезом и отложением коллагена в органах и тканях. Описана больная ювенильной системной склеродермией с индурацией кожи и подлежащих тканей, а также стойкими контрактурами крупных суставов с детского возраста. На этом клиническом примере рассматриваются особенности течения и дифференциальная диагностика системной и ограниченной (очаговой) склеродермии и склеродермоподобных состояний у пациентов детского возраста. Показаны возможности подбора патогенетической терапии, направленной на улучшение качества жизни пациентов с уже сформированным фенотипом заболевания.

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.

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The authors declare that there are no conflicts of interest present.