| Abstract [eng] |
Background. Localized scleroderma is a rare chronic autoimmune inflammatory disorder, belonging to the spectrum of sclerosing skin diseases, affecting patients of any age, more frequently Caucasian females. Localized scleroderma affects patients' daily quality of life. The risk of developing the disease, pathogenetic mechanisms and etiology remain poorly understood and are insufficiently described in the scientific literature. According to literature, diagnosis is based on clinical evaluation, laboratory and instrumental diagnostic tests, however there is no universal consensus on the treatment. Various therapeutic approaches are proposed – topical, immunomodulatory, systemic immunosuppressive, phototherapy and physical medicine – in practice, the choice of treatment largely depends on the specialist. This work aims to describe the mechanisms of the disease and to provide latest scientific data on presentation, diagnosis, course, treatment and management of localized scleroderma to prevent the progression of the disease. Clinical case. This work presents a case of juvenile localized scleroderma in a seven-year-old female treated at Vilnius University Hospital Santaros Clinics. The patient was diagnosed with localized scleroderma after clinical evaluation and laboratory diagnostics, both systemic and topical treatments were administered, resulting in regression of skin lesions. Active treatment was administered for four years before disease remission was achieved. Patient is currently under follow-up during remission. Literature review. Scientific literature on localized scleroderma was selected and analyzed. The review focused on prevalence, etiology, pathogenesis, classification, diagnostics, symptoms and assessment, treatment. Objective. To present a clinical case of localized scleroderma and to provide an overview of current scientific literature on localized scleroderma. Methods. Search was conducted electronically using international databases “PubMed”, “Google scholar”, “Cochrane Library”, keywords and active filters. Keywords: “localized scleroderma”, “morphea”, “juvenile localized scleroderma”, in combination with: “extracutaneous involvement”, “treatment”, “diagnosis”, “imaging”. Articles published in English from 2015 to 2025 were selected and analyzed, preference given to those published within the last five years. Additional older but methodologically relevant sources were included. 366 publications were reviewed in total, of which 150 were selected for full-text review. 52 publications were used for the literature review. Clinical case description was based on retrospectively collected data from outpatient and inpatient visits, obtained with written parental consent (Appendix No. 1). The case was analyzed in accordance with principles of professional ethics. Clinical data was compared with recent scientific literature. Results. The analysis of the clinical case and scientific literature allowed the systematization of information on localized scleroderma, presenting latest scientific insights. Conclusions. Localized scleroderma is a rare, autoimmune disease with significant clinical heterogeneity and poorly understood pathogenesis. Classification remains complex due to overlapping types and unclear distinctions between them. Diagnosis is typically based on clinical evaluation, as laboratory and instrumental assessments have limited prognostic value and are rarely applied in clinical practice. Treatment depends on physician specialization and resource availability. Topical corticosteroids and phototherapy are commonly used, while systemic therapy is recommended for rapidly progressing or widespread forms. First-line agent for systemic treatment is methotrexate, often administered together with systemic corticosteroids. To ensure timely diagnosis and effective treatment – particularly in the pediatric population – standardized diagnostic and treatment guidelines and interdisciplinary collaboration is essential. |
