title: Juvenile localized scleroderma: clinical and epidemiological features in 750 children. An international study.
abstract: OBJECTIVE
  Juvenile localized scleroderma (JLS) includes a number of conditions often grouped together. With the long-term goal of developing uniform classification criteria, we studied the epidemiological, clinical and immunological features of children with JLS followed by paediatric rheumatology and dermatology centres.
  
  
  METHODS
  A large, multicentre, multinational study was conducted by collecting information on the demographics, family history, triggering environmental factors, clinical and laboratory features, and treatment of patients with JLS.
  
  
  RESULTS
  Seven hundred and fifty patients with JLS from 70 centres were enrolled into the study. The disease duration at diagnosis was 18 months. Linear scleroderma (LS) was the most frequent subtype (65%), followed by plaque morphea (PM) (26%), generalized morphea (GM) (7%) and deep morphea (DM) (2%). As many as 15% of patients had a mixed subtype. Ninety-one patients (12%) had a positive family history for rheumatic or autoimmune diseases; 100 (13.3%) reported environmental events as possible trigger. ANA was positive in 42.3% of the patients, with a higher prevalence in the LS-DM subtype than in the PM-GM subtype. Scl70 was detected in the sera of 3% of the patients, anticentromere antibody in 2%, anti-double-stranded DNA in 4%, anti-cardiolipin antibody in 13% and rheumatoid factor in 16%. Methotrexate was the drug most frequently used, especially during the last 5 yr.
  
  
  CONCLUSIONS
  This study represents the largest collection of patients with JLS ever reported. The insidious onset of the disease, the delay in diagnosis, the recognition of mixed subtype and the better definition of the other subtypes should influence our efforts in educating trainees and practitioners and help in developing a comprehensive classification system for this syndrome.
authors.affiliation: Dipartimento di Pediatria, Università di Padova, Via Giustiniani 3, 35128 Padova, Italy. zulian@pediatria.unipd.it
authors.forename: F
authors.initials: F
authors.lastname: Zulian
authors.forename: B H
authors.initials: BH
authors.lastname: Athreya
authors.forename: R
authors.initials: R
authors.lastname: Laxer
authors.forename: A M
authors.initials: AM
authors.lastname: Nelson
authors.forename: S K
authors.initials: SK
authors.lastname: Feitosa de Oliveira
authors.forename: M G
authors.initials: MG
authors.lastname: Punaro
authors.forename: R
authors.initials: R
authors.lastname: Cuttica
authors.forename: G C
authors.initials: GC
authors.lastname: Higgins
authors.forename: L W A
authors.initials: LW
authors.lastname: Van Suijlekom-Smit
authors.forename: T L
authors.initials: TL
authors.lastname: Moore
authors.forename: C
authors.initials: C
authors.lastname: Lindsley
authors.forename: J
authors.initials: J
authors.lastname: Garcia-Consuegra
authors.forename: M O
authors.initials: MO
authors.lastname: Esteves Hilário
authors.forename: L
authors.initials: L
authors.lastname: Lepore
authors.forename: C A
authors.initials: CA
authors.lastname: Silva
authors.forename: C
authors.initials: C
authors.lastname: Machado
authors.forename: S M
authors.initials: SM
authors.lastname: Garay
authors.forename: Y
authors.initials: Y
authors.lastname: Uziel
authors.forename: G
authors.initials: G
authors.lastname: Martini
authors.forename: I
authors.initials: I
authors.lastname: Foeldvari
authors.forename: A
authors.initials: A
authors.lastname: Peserico
authors.forename: P
authors.initials: P
authors.lastname: Woo
authors.forename: J
authors.initials: J
authors.lastname: Harper
mesh_terms: D000293:Adolescent; D017668:Age of Onset; D001323:Autoantibodies / Q000097:blood; D001327:Autoimmune Diseases / Q000235:genetics; D002648:Child; D002675:Child, Preschool; D004777:Environment; D005260:Female; D020022:Genetic Predisposition to Disease; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007223:Infant; D007231:Infant, Newborn; D007391:International Cooperation; D008297:Male; D008727:Methotrexate / Q000627:therapeutic use; D012216:Rheumatic Diseases / Q000235:genetics; D012307:Risk Factors; D012594:Scleroderma, Localized / Q000175:diagnosis* / Q000188:drug therapy / Q000453:epidemiology / Q000209:etiology
chemical_list: D001323:Autoantibodies; D007166:Immunosuppressive Agents; D008727:Methotrexate