Juvenile Localized Scleroderma with Cutaneous and Extracutaneous Involvement: Long-Term Observational Outcomes from a...

Area of Science:

• Pediatric Rheumatology
• Dermatology
• Immunology

Background:

• Juvenile localized scleroderma (JLS) is a chronic autoimmune condition affecting children.
• Understanding JLS characteristics, extracutaneous involvement (ECI), and treatment response is vital for patient management.

Purpose of the Study:

• To characterize JLS patients, including demographics, clinical presentation, and laboratory findings.
• To identify the prevalence and types of ECI in JLS.
• To evaluate treatment responses to conventional disease-modifying antirheumatic drugs (cDMARDs).

Main Methods:

• Retrospective single-center cohort study of 87 JLS patients (2015-2025).
• Inclusion criteria: Padua classification criteria and ≥6 months follow-up.
• Systematic collection of clinical, laboratory, and treatment data; ECI analysis via comparative statistics.

Main Results:

• 77% of patients were female; mean age at onset was 7.1 years, with a 1.2-year diagnostic delay.
• Linear scleroderma was the most frequent subtype (36.7%); lower extremities were most affected (39.7%).
• ECI occurred in 47.1%, primarily musculoskeletal, linked to severe subtypes and poorer cDMARD response. 85.9% responded to cDMARDs, with methotrexate showing high efficacy.

Conclusions:

• JLS presents significant cutaneous and extracutaneous morbidity, particularly in generalized/pansclerotic subtypes with limb involvement.
• Early recognition and systematic ECI assessment are crucial.
• Longitudinal monitoring in specialized centers and further research are needed to optimize treatment for refractory cases.