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

OBJECTIVE

To describe demographic, clinical, and laboratory characteristics of patients with juvenile localized scleroderma (JLS), determine the presence of extracutaneous involvement (ECI) and other comorbidities, and assess treatment response.

MATERIALS AND

METHODS

Retrospective single-center cohort study of JLS patients followed between 2015 and 2025 who met Padua classification criteria and had ≥6 months of regular follow-up. Clinical, laboratory, and treatment data were systematically collected, and ECI was analyzed using comparative statistics.

RESULTS

Among 87 patients, 77% were female. Mean ages at symptom onset, diagnosis, and last visit were 7.1, 9.0, and 14.4 years, respectively, with a median diagnostic delay of 1.2 years. The most frequent subtype was linear scleroderma (36.7%), followed by circumscribed (31.0%), generalized (20.6%), mixed-type (6.8%), and pansclerotic morphea (4.6%). The lower extremities were the most commonly affected, accounting for 54 of 136 sites (39.7%). The ECI occurred in 47.1% of patients, mainly musculoskeletal, and was linked to generalized/pansclerotic subtypes, limb involvement, higher erythrocyte sedimentation rate, higher disease activity scores, and poorer response to conventional disease-modifying antirheumatic drugs (cDMARDs). Overall, 85 patients received at least 1 cDMARD, with 85.9% responding. Methotrexate regimens had high response rates; 14.1% were non-responsive to at least 2 cDMARDs, and 5.7% had active disease at last visit.

CONCLUSION

The JLS carries substantial cutaneous and extracutaneous morbidity, particularly in generalized and pansclerotic subtypes with limb involvement. These findings underscore the importance of early recognition, systematic assessment of ECI, and close longitudinal monitoring in specialized centers and support the need for prospective studies to refine risk stratification and optimize treatment strategies for refractory JLS.