Specific clinical features that may help distinguish and measure disease activity for localized scleroderma in children have been identified, according to results of a study published in the Journal of Rheumatology.

Researchers conducted a prospective multicenter study that included 90 children with active or inactive localized scleroderma skin lesions. Clinical features of these lesions were analyzed for common characteristics that could help define their specificity and significance in measuring disease activity. Physician’s global assessments of activity (PGA-A) was used to correlate clinical lesion features with disease activity.

After statistical analysis, the investigators found that linear scleroderma was the most common disease subtype. Several clinical features specific for disease activity were identified, including violaceous color, tactile warmth, abnormal skin texture, and erythema. The researchers also determined that skin thickening correlated with PGA-A scores for active lesions.


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The primary study limitation was the lack of a gold-standard measurement tool for disease activity.

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“Our study provides important information for clinicians caring for patients with pediatric [localized scleroderma], including the lack of a universal activity feature and lack of specificity of skin thickness for active disease. These results will help guide development of a sensitive, responsive activity tool to improve care of patients with [localized scleroderma],” the researchers wrote.

Further studies are needed to fully understand how to best use clinical features to measure disease activity in localized scleroderma.

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Reference

Li SC, Li X, Pope E, et al. New features for measuring disease activity in pediatric localized scleroderma [published online September 9, 2018]. J Rheumatol. doi:10.3899/jrheum.171381