Features of the course of and diagnostics of juvenile localized scleroderma: analysis of clinical cases

Abstract

Juvenile localized scleroderma is characterized by foci of atrophic skin and lying beneath tissues, but damage of deep soft tissues, bones and joints can be observed, which, in addition to cosmetic changes, can lead to functional disorders and pain. A comparison of literature data and personal observations about the features of the onset, the development of clinical symptoms, the course, results of laboratory and instrumental examinations in children with localized scleroderma was carried out.

Purpose - analyze your own data on the diagnosis and course of the pathological process in patients with localized form of juvenile scleroderma and based on the latest data from literary sources, we compare the own data with those from modern literature in the diagnosis of this rare disease.

Materials and methods. We observed 48 patients with juvenile scleroderma for the period 2010-2020. The ultrasound examination of blood vessels, ultrasound examination of the abdominal cavity organs, heart and joints, X-ray examination of the lungs and joints, electrocardiographic examination, MRI examination and the indices of autoimmune activity were determined.

Results. The features of the course and diagnosis of juvenile localized scleroderma have been analyzed, namely, local skin lesions in the form of multiple foci with later development of sclerosis and atrophy are observed, so as Raynaud’s syndrome, articular syndrome in the form of arthralgias. When conducting laboratory studies, indicators of general inflammatory activity in localized scleroderma are informative. Ultrasound examination of the skin and muscles versus traditional traumatic biopsy is an informative method for diagnosing changes in the dermis, subcutaneous tissue, soft tissues and blood vessels, which is a sign of active scleroderma.

Conclusions. Juvenile localized scleroderma has definite features of the clinical course, it is important to bear in mind when establishing the diagnosis: local skin lesion in the form of multiple foci with later development of sclerosis and atrophy, Raynaud’s syndrome, articular syndrome in the form of arthralgias, uninformative laboratory data, visceral damages are frequently manifested by functional disorders, unevenness pathological process in vessels with changes primarily in the intima-media complex, informative ultrasound examination of the skin and muscles.

The research was carried out in accordance with the principles of the Helsinki declaration. The study protocol was approved by the Local ethics committee of all participating institutions. The informed consent of the patient was obtained for conducting the studies.

No conflict of interests was declared by the authors.