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Ocular involvement in children with localized scleroderma: a multicenter study
  1. Maria Elisabetta Zannin (ezannin{at},
  2. Giorgia Martini,
  3. Balu H Athreya,
  4. Ricardo Russo,
  5. Gloria C Higgins,
  6. Fabio Vittadello,
  7. Maria Giannina Alpigiani,
  8. Mariolina Alessio,
  9. Mauro Paradisi,
  10. Patricia Woo,
  11. Francesco Zulian
  1. Department of Paediatrics, University of Padua, Italy
  2. Department of Paediatrics, University of Padua, Italy
  3. A.I. Du Pont Hospital for Children, Wilmington, United States
  4. Hospital de Pediatria Juan P. Garrahan, Buenos Aires, Argentina
  5. Children's Hospital, Columbus, United States
  6. Department of Paediatrics, University of Padua, Italy
  7. G. Gaslini Institute, Genoa, Italy
  8. University Federico II, Naples, Italy
  9. Istituto Dermatopatico dell'Immacolata, IRCCS, Rome, Italy
  10. Great Ormond Street Hospital, London, United Kingdom
  11. Department of Paediatrics, University of Padua, Italy


    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.

    • Localized scleroderma
    • eye
    • morphea
    • ocular involvement
    • scleroderma en coup de sabre

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