Juvenile Localized Scleroderma from a Pediatric Rheumatology Perspective: A Single-Center Experience
Juvenile Localized Scleroderma
Keywords:Juvenile localized scleroderma, morphea, treatment
Aim: To evaluate juvenile localized scleroderma, which is a disease with high rates of cosmetic and functional sequelae in children, from a pediatric rheumatology perspective.
Materials and Methods: We retrospectively investigated the data of patients who were diagnosed with juvenile localized scleroderma (JLS) in our pediatric rheumatology clinic between 2015 and 2022, were aged <18 years and attended their follow-ups regularly. Demographic, clinical, treatment-related, and prognostic data of the patients were included.
Results: Among the 19 patients diagnosed with JLS, 12 (63.2%) were female, and 7 (36.8%) were male. The female-to-male ratio in the sample was 1.7. Eight (42.1%) patients had circumscribed JLS, 8 (42.1%) had linear JLS, 2 (10.6%) had mixed JLS, and 1 (5.3%) had generalized JLS. The mean age of onset of symptoms in the patients was 8.2±5.5 years, while their mean age of diagnosis was 9.4±4.9 years. The most frequently involved anatomical regions were the extremities, whose involvement was found in 15 (78.9%) patients. The prevalence of lesions crossing joints was 57.9%, and joint damage was seen in 21.1% of the patients. The rate of cosmetic sequelae was 73.7%. There was antinuclear antibody positivity in 52.6% of the patients. Systemic involvement did not occur in any patients during their follow-ups. The most frequently used treatment agent was methotrexate. Complete remission was achieved in 2 (10.6%) patients.
Conclusion: As it can lead to high degrees of cosmetic and functional sequelae, it is needed to diagnose juvenile localized scleroderma early and start an aggressive treatment in the early period. To avoid wasting time, it is important, especially for pediatricians, to immediately order biopsies from suspected lesions or refer these patients to pediatric rheumatology clinics.
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