PT  - JOURNAL ARTICLE
AU  - Maria Elisabetta Zannin
AU  - Giorgia Martini
AU  - Balu H Athreya
AU  - Ricardo Russo
AU  - Gloria Higgins
AU  - Fabio Vittadello
AU  - Maria Giannina Alpigiani
AU  - Mariolina Alessio
AU  - Mauro Paradisi
AU  - Patricia Woo
AU  - Francesco Zulian
TI  - Ocular involvement in children with localised scleroderma: a multi-centre study
AID  - 10.1136/bjo.2007.116038
DP  - 2007 Oct 01
TA  - British Journal of Ophthalmology
PG  - 1311--1314
VI  - 91
IP  - 10
4099  - http://bjo.bmj.com/content/91/10/1311.short
4100  - http://bjo.bmj.com/content/91/10/1311.full
SO  - Br J Ophthalmol2007 Oct 01; 91
AB  - 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.Conclusion: 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.