title: Ocular involvement in children with localised scleroderma: a multi-centre study.
abstract: BACKGROUND
  Most of the available documentation in the literature on ocular involvement in localised scleroderma (LS) are descriptions of single cases in adult patients. This article reports the frequency and specific features of ocular involvement in a large cohort of children with juvenile LS (JLS).
  
  
  METHODS
  Data from a large, multi-centre, multinational study of children with LS were used to collect and analyse specific information on ocular involvement.
  
  
  RESULTS
  24 out of 750 patients (3.2%) revealed a significant ocular involvement. 16 were female and 8 male. 16 patients (66.7%) had scleroderma "en coup de sabre" (ECDS) of the face, 5 (20.8%) had the linear subtype, 2 (8.3%) had generalised morphea (GM) and one (4.2%) had plaque morphea (PM). Of the 24 patients with eye involvement, 10 patients (41.7%) reported adnexa (eyelids and eyelashes) abnormalities, 7 (29.2%) anterior segment inflammation (5 anterior uveitis, 2 episcleritis) and 3 central nervous system-related abnormalities. 4 patients presented single findings such as paralytic strabismus (1), pseudopapilloedema (1) and refractive errors (2). Other extracutaneous manifestations were detected in a significantly higher number of patients with ocular involvement and were mostly neurological.
  
  
  CONCLUSIONS
  Ocular abnormalities are not unusual in patients with JLS, especially in the ECDS subtype. They are frequently associated with other internal organ involvement, particularly the central nervous system (CNS). Careful ophthalmic monitoring is recommended for every patient with JLS, but is mandatory in those with skin lesions on the face and/or concomitant CNS involvement.
authors.affiliation: Department of Pediatrics, Via Giustiniani 3, 35128 Padova, Italy. ezannin@pediatria.unipd.it
authors.forename: Maria Elisabetta
authors.initials: ME
authors.lastname: Zannin
authors.forename: Giorgia
authors.initials: G
authors.lastname: Martini
authors.forename: Balu H
authors.initials: BH
authors.lastname: Athreya
authors.forename: Ricardo
authors.initials: R
authors.lastname: Russo
authors.forename: Gloria
authors.initials: G
authors.lastname: Higgins
authors.forename: Fabio
authors.initials: F
authors.lastname: Vittadello
authors.forename: Maria Giannina
authors.initials: MG
authors.lastname: Alpigiani
authors.forename: Mariolina
authors.initials: M
authors.lastname: Alessio
authors.forename: Mauro
authors.initials: M
authors.lastname: Paradisi
authors.forename: Patricia
authors.initials: P
authors.lastname: Woo
authors.forename: Francesco
authors.initials: F
authors.lastname: Zulian
mesh_terms: D000293:Adolescent; D017677:Age Distribution; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D005128:Eye Diseases / Q000150:complications* / Q000453:epidemiology; D005140:Eyelashes / Q000002:abnormalities; D005143:Eyelids / Q000002:abnormalities; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D015423:Scleritis / Q000150:complications / Q000453:epidemiology; D012594:Scleroderma, Localized / Q000150:complications* / Q000453:epidemiology; D017678:Sex Distribution; D014606:Uveitis, Anterior / Q000150:complications / Q000453:epidemiology