title: Localized scleroderma in childhood is not just a skin disease.
abstract: OBJECTIVE
  Juvenile localized scleroderma is usually considered a disease that is confined to the skin and subcutaneous tissue. We studied the prevalence and clinical features of extracutaneous manifestations in a large cohort of children with juvenile localized scleroderma.
  
  
  METHODS
  Data from a multinational study on juvenile scleroderma was used for this in-depth study. Clinical features of patients with extracutaneous manifestations were compared with those of patients who had exclusively skin involvement.
  
  
  RESULTS
  Seven hundred fifty patients entered the study. One hundred sixty-eight patients (22.4%) presented with a total of 193 extracutaneous manifestations, as follows: articular (47.2%), neurologic (17.1%), vascular (9.3%), ocular (8.3%), gastrointestinal (6.2%), respiratory (2.6%), cardiac (1%), and renal (1%). Other autoimmune conditions were present in 7.3% of patients. Neurologic involvement consisted of epilepsy, central nervous system vasculitis, peripheral neuropathy, vascular malformations, headache, and neuroimaging abnormalities. Ocular manifestations were episcleritis, uveitis, xerophthalmia, glaucoma, and papilledema. In more than one-fourth of these children, articular, neurologic, and ocular involvements were unrelated to the site of skin lesions. Raynaud's phenomenon was reported in 16 patients. Respiratory involvement consisted essentially of restrictive lung disease. Gastrointestinal involvement was reported in 12 patients and consisted exclusively of gastroesophageal reflux. Thirty patients (4%) had multiple extracutaneous features, but systemic sclerosis (SSc) developed in only 1 patient. In patients with extracutaneous involvement, the prevalence of antinuclear antibodies and rheumatoid factor was significantly higher than that among patients with only skin involvement. However, Scl-70 and anticentromere, markers of SSc, were not significantly increased.
  
  
  CONCLUSIONS
  Extracutaneous manifestations of juvenile localized scleroderma developed in almost one-fourth of the children in this study. These extracutaneous manifestations often were unrelated to the site of the skin lesions and sometimes were associated with multiple organ involvement. The risk of developing SSc was very low. This subgroup of patients with juvenile localized scleroderma should be evaluated extensively, treated more aggressively, and monitored carefully.
authors.affiliation: Dipartimento di Pediatria, Università di Padova, Padua, Italy. zulian@pediatria.unipd.it
authors.forename: Francesco
authors.initials: F
authors.lastname: Zulian
authors.forename: Cristina
authors.initials: C
authors.lastname: Vallongo
authors.forename: Patricia
authors.initials: P
authors.lastname: Woo
authors.forename: Ricardo
authors.initials: R
authors.lastname: Russo
authors.forename: Nicolino
authors.initials: N
authors.lastname: Ruperto
authors.forename: John
authors.initials: J
authors.lastname: Harper
authors.forename: Graciela
authors.initials: G
authors.lastname: Espada
authors.forename: Fabrizia
authors.initials: F
authors.lastname: Corona
authors.forename: Masha
authors.initials: M
authors.lastname: Mukamel
authors.forename: Richard
authors.initials: R
authors.lastname: Vesely
authors.forename: Elzbieta
authors.initials: E
authors.lastname: Musiej-Nowakowska
authors.forename: Jeff
authors.initials: J
authors.lastname: Chaitow
authors.forename: Joan
authors.initials: J
authors.lastname: Ros
authors.forename: Maria T
authors.initials: MT
authors.lastname: Apaz
authors.forename: Valeria
authors.initials: V
authors.lastname: Gerloni
authors.forename: Henryka
authors.initials: H
authors.lastname: Mazur-Zielinska
authors.forename: Susan
authors.initials: S
authors.lastname: Nielsen
authors.forename: Susanne
authors.initials: S
authors.lastname: Ullman
authors.forename: Gerd
authors.initials: G
authors.lastname: Horneff
authors.forename: Carine
authors.initials: C
authors.lastname: Wouters
authors.forename: Giorgia
authors.initials: G
authors.lastname: Martini
authors.forename: Rolando
authors.initials: R
authors.lastname: Cimaz
authors.forename: Ronald
authors.initials: R
authors.lastname: Laxer
authors.forename: Balu H
authors.initials: BH
authors.lastname: Athreya
mesh_terms: D001327:Autoimmune Diseases / Q000150:complications / Q000175:diagnosis / Q000453:epidemiology; D002648:Child; D015331:Cohort Studies; D005128:Eye Diseases / Q000150:complications / Q000175:diagnosis / Q000453:epidemiology; D005260:Female; D005767:Gastrointestinal Diseases / Q000150:complications / Q000175:diagnosis / Q000453:epidemiology; D006331:Heart Diseases / Q000150:complications / Q000175:diagnosis / Q000453:epidemiology; D006801:Humans; D007592:Joint Diseases / Q000150:complications / Q000175:diagnosis / Q000453:epidemiology; D007674:Kidney Diseases / Q000150:complications / Q000175:diagnosis / Q000453:epidemiology; D008297:Male; D009422:Nervous System Diseases / Q000150:complications / Q000175:diagnosis / Q000453:epidemiology; D015995:Prevalence; D012140:Respiratory Tract Diseases / Q000150:complications / Q000175:diagnosis / Q000453:epidemiology; D012189:Retrospective Studies; D012594:Scleroderma, Localized / Q000150:complications* / Q000175:diagnosis / Q000453:epidemiology; D014652:Vascular Diseases / Q000150:complications / Q000175:diagnosis / Q000453:epidemiology