Clinical characteristics of juvenile localized scleroderma
Objective To analyze the clinical manifestations,laboratory results and treatment outcomes of juvenile localized scleroderma(JLS).Methods This was a retrospective case series study.Epidemiological and clinical data of patients with JLS treated in the Department of Dermatology,Beijing Children's Hospital,Capital Medical University from January 2019 to August 2021 were analyzed.Results Among the 228 children,there were 95 males and 133 females,with a male-to-female ratio of 1.0:1.4.The median age of onset was 67 months(5.6 years),and the time from onset to diagnosis was 1 month to 106 months.Eight patients(3.5％)had suspected predisposing factors before onset,25 patients(11.0％)had other diseases at the same time,and 3 patients(1.3％)had a family history of autoimmune diseases.The most common clinical type of JLS was circumscribed morphea(101 cases,44.3％),followed by linear scleroderma(91 cases,39.9％),generalized morphea(31 cases,13.6％),mixed scleroderma(23 cases,10.1％)and pansclerotic morphea(2 cases,0.9％).Among them,76 patients(46.3％)were antinuclear antibody(ANA)positive,and 14 patients(15.0％)were extractable nuclear antigen antibody(ENA)positive.Ninety-one of patients with circumscribed morphea were treated with medium and strong corticosteroids,vitamin D3 derivatives or calcineurin inhibitors.Patients of other types received systemic corticosteroid treatment,with an initial dose range of 1-2 mg/kg and a maximum dose of 60 mg/d.Among them,72 patients were additionally treated with Methotrexate,with an initial dose range of 10-15 mg/m2,once a week,and 9 patients were additionally treated with biological agents.The follow-up results showed that the skin symptoms of the patients who were followed up in the dermatology outpatient department had improved to a certain extent and could remain inactive.Conclusions Children with JLS in the dermatology department are mainly preschool-and school-age.Circumscribed morphea is the most common type,mainly treated with glucocorticoids,vitamin D3 derivatives or calcineurin inhibitors.No specific laboratory test index is found.Corticosteroids combined with Methotrexate are recommended for systematic treatment of other types of JLS.
NETL
NSTL
万方数据
