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Ocular involvement in children with localised scleroderma: a multi-centre study
  1. Maria Elisabetta Zannin1,
  2. Giorgia Martini1,
  3. Balu H Athreya2,
  4. Ricardo Russo3,
  5. Gloria Higgins4,
  6. Fabio Vittadello1,
  7. Maria Giannina Alpigiani5,
  8. Mariolina Alessio6,
  9. Mauro Paradisi7,
  10. Patricia Woo8,
  11. Francesco Zulian1
  1. 1
    Department of Pediatrics, Padova, Italy
  2. 2
    AI Du Pont Hospital for Children, Wilmington, DE, USA
  3. 3
    Hospital de Pediatria Juan P Garrahan, Buenos Aires, Argentina
  4. 4
    Children’s Hospital, Columbus, OH, USA
  5. 5
    Pediatric Division, “G. Gaslini” Institute, Genoa, Italy
  6. 6
    Department of Pediatrics, University “Federico II”, Naples, Italy
  7. 7
    Istituto Dermatopatico dell’Immacolata, IRCCS, Rome
  8. 8
    Great Ormond Street Hospital, London, UK
  1. Maria Elisabetta Zannin, Department of Pediatrics, Via Giustiniani 3, 35128 Padova, Italy; ezannin{at}pediatria.unipd.it

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.

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.

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Footnotes

  • Competing interests: None declared.

  • Abbreviations:
    ACL

    anticardiolipin antibodies

    ANA

    antinuclear antibodies

    AU

    anterior uveitis

    CNS

    central nervous system

    DM

    deep morphea

    ECDS

    en coup de sabre

    GM

    generalised morphea

    JLS

    juvenile localised scleroderma

    LS

    localised scleroderma

    PM

    plaque morphea

    RF

    rheumatoid factor

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