In Localized Scleroderma, New Features Can Determine Disease Activity Among Children

Lesion features such as erythema and violaceous color can help determine whether a child has active localized scleroderma; however, skin thickening is not one of those features, according to findings from a prospective study.


“These results will help guide development of a sensitive, responsive activity tool to improve care of patients with localized scleroderma,” the researchers wrote.


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To reach this conclusion, the researchers evaluated data on 103 participants with active and inactive localized scleroderma skin lesions from across multiple centers. All of the participants had a similar age of disease onset, gender, and disease patterns.


At each of 3 visits, a standardized evaluation of a single designated study lesion per participant was performed. The researchers then evaluated the pattern and correlation between assessed features and physician’s global assessments of activity.


Of the 103 participants, 90 had data that the researchers could evaluate. Of those 90 participants, 66 had active disease and 24 had inactive disease.


The most common subtype was linear scleroderma. Erythema, violaceous color, tactile warmth, abnormal skin texture, and disease extension were the features specific for this active disease.


Scores for these variables changed over time and correlated with physician’s global assessments of activity of the lesion. However, the presence or level of skin thickeningeither of lesion edge or centerwas not enough to distinguish active and inactive lesions. At the same time, in active lesions, skin thickening scores did correlate with physician’s global assessments of activity scores.


According to regression analysis, the combination of erythema, disease extension, violaceous color, skin thickening, and abnormal texture was predictive of physician’s global assessments of activity at study entry. Damage features were common regardless of activity status.


—Colleen Murphy



Li SC, Li X, Pope E, et al. New features for measuring disease activity in pediatric localized scleroderma. J Rheumatol. 2018;45(12):1680-1688.