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. 2011 Oct;50(10):1865-8.
doi: 10.1093/rheumatology/ker142. Epub 2011 Jul 5.

Clinical features of childhood localized scleroderma in an incidence cohort

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Clinical features of childhood localized scleroderma in an incidence cohort

Ariane L Herrick et al. Rheumatology (Oxford). 2011 Oct.

Abstract

Objectives: Our aim was to describe clinical features and pattern of care in children with localized scleroderma presenting to secondary care during a 25-month incidence study.

Methods: Eighty-seven patients were identified, and clinical features, serum autoantibodies, current treatment and outcome at 12 months were documented.

Results: Fifty-eight (67%) had linear scleroderma, 25 (29%) non-linear morphoea and 4 (4%) a mixed pattern. Of the 58 patients with linear scleroderma, 29 (50%) presented with lesions of the trunk and/or limbs only, 26 (45%) with face-head localization only and 3 (5%) with both. Thirteen (15%) had extracutaneous features and 16 (43%) out of 37 were ANA positive. At 12 months, 59% were on MTX. At 12 months, 51 (65%) were improved/resolved, 14 (18%) were unchanged and 13 (17%) had deteriorated.

Conclusion: Key findings included the high prevalence of face-head involvement in those with linear disease, and the high prevalence of extracutaneous disease and of ANA positivity. After 12 months, most patients improved according to clinician's opinion.

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