Orphanet Journal of Rare Diseases (Sep 2020)

Management of ocular involvement in the acute phase of Stevens-Johnson syndrome and toxic epidermal necrolysis: french national audit of practices, literature review, and consensus agreement

  • D. Thorel,
  • S. Ingen-Housz-Oro,
  • G. Royer,
  • A. Delcampe,
  • N. Bellon,
  • C. Bodemer,
  • A. Welfringer-Morin,
  • D. Bremond-Gignac,
  • M. P. Robert,
  • M. Tauber,
  • F. Malecaze,
  • O. Dereure,
  • V. Daien,
  • A. Colin,
  • C. Bernier,
  • C. Couret,
  • B. Vabres,
  • F. Tetart,
  • B. Milpied,
  • T. Cornut,
  • B. Ben Said,
  • C. Burillon,
  • N. Cordel,
  • L. Beral,
  • N. de Prost,
  • P. Wolkenstein,
  • M. Muraine,
  • J. Gueudry

DOI
https://doi.org/10.1186/s13023-020-01538-x
Journal volume & issue
Vol. 15, no. 1
pp. 1 – 7

Abstract

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Abstract Stevens-Johnson Syndrome (SJS) and toxic epidermal necrolysis (TEN) can lead to severe ophthalmologic sequelae. The main risk factor is the severity of the initial ocular involvement. There are no recommendations for ocular management during acute phase. We conducted a national audit of current practice in the 11 sites of the French reference center for toxic bullous dermatoses and a review of the literature to establish therapeutic consensus guidelines. We sent a questionnaire on ocular management practices in SJS/ TEN during acute phase to ophthalmologists and dermatologists. The survey focused on ophthalmologist opinion, pseudomembrane removal, topical ocular treatment (i.e. corticosteroids, antibiotics, antiseptics, artificial tear eye drops, vitamin A ointment application), amniotic membrane transplantation, symblepharon ring use, and systemic corticosteroid therapy for ophthalmologic indication. Nine of 11 centers responded. All requested prompt ophthalmologist consultation. The majority performed pseudomembrane removal, used artificial tears, and vitamin A ointment (8/9, 90%). Combined antibiotic-corticosteroid or corticosteroid eye drops were used in 6 centers (67%), antibiotics alone and antiseptics in 3 centers (33%). Symblepharon ring was used in 5 centers (55%) if necessary. Amniotic membrane transplantation was never performed systematically and only according to the clinical course. Systemic corticosteroid therapy was occasionally used (3/9, 33%) and discussed on a case-by-case basis. The literature about ocular management practice in SJS/ TEN during acute phase is relatively poor. The role of specific treatments such as local or systemic corticosteroid therapy is not consensual. The use of preservatives, often present in eye drops and deleterious to the ocular surface, is to be restricted. Early amniotic membrane transplantation seems to be promising.

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