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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.

Original publication

DOI

10.1093/rheumatology/ker142

Type

Journal article

Journal

Rheumatology (Oxford, England)

Publication Date

10/2011

Volume

50

Pages

1865 - 1868

Addresses

Arthritis Research UK Epidemiology Unit, University of Manchester, Stopford Building, Oxford Road, Manchester M13 9PT, UK. ariane.herrick@manchester.ac.uk

Keywords

Back, Extremities, Face, Skin, Humans, Scleroderma, Localized, Methotrexate, Immunosuppressive Agents, Severity of Illness Index, Questionnaires, Cohort Studies, Child, Female, Male