RT Journal Article
SR Electronic
T1 Ocular involvement in children with localized scleroderma: a multicenter study
JF British Journal of Ophthalmology
JO Br J Ophthalmol
FD BMJ Publishing Group Ltd.
DO 10.1136/bjo.2007.116038
A1 Maria Elisabetta Zannin
A1 Giorgia Martini
A1 Balu H Athreya
A1 Ricardo Russo
A1 Gloria C Higgins
A1 Fabio Vittadello
A1 Maria Giannina Alpigiani
A1 Mariolina Alessio
A1 Mauro Paradisi
A1 Patricia Woo
A1 Francesco Zulian
YR 2007
UL http://bjo.bmj.com/content/early/2007/05/02/bjo.2007.116038.abstract
AB Background: Most of the available documentation in the literature on ocular involvement in Localized Scleroderma (LS) is description of single cases in adult patients. We report the frequency and specific features of ocular involvement in a large cohort of children with Juvenile LS (JLS). Methods: Data from a large, multicenter, multinational study of children with JLS was used, to collect and analyze specific information on ocular involvement. Results: Twenty-four out of 750 patients (3.2%) revealed a significant ocular involvement. Sixteen were female and 8 male. Sixteen patients (66.7%) had scleroderma "En coup de sabre"(ECDS) of the face, 5 (20.8%) had the linear subtype (LS), 2 (8.3%) had generalized 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, seven (29.2%) anterior segment inflammation (5 anterior uveitis, 2 episcleritis) and 3 central nervous system-related abnormalities. Four patients presented single findings such as paralytic strabismus (1), pseudopapilledema (1), and refractive errors (2). Other extra-cutaneous manifestations, mostly neurological, were detected in a significantly higher number of patients with ocular involvement. 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 nervouse system. A careful ophthalmic monitoring is recommended for every patient with JLS, but is mandatory in those with skin lesions on the face and/or concomitant neurological involvement.