Ocular involvement in children with localised scleroderma: a multi-centre study.
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.
Zannin
Maria Elisabetta
ME
Department of Pediatrics, Via Giustiniani 3, 35128 Padova, Italy. ezannin@pediatria.unipd.it
Martini
Giorgia
G
Athreya
Balu H
BH
Russo
Ricardo
R
Higgins
Gloria
G
Vittadello
Fabio
F
Alpigiani
Maria Giannina
MG
Alessio
Mariolina
M
Paradisi
Mauro
M
Woo
Patricia
P
Zulian
Francesco
F
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