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Article Dans Une Revue Orphanet Journal of Rare Diseases Année : 2023

Ocular sequelae of epidermal necrolysis: French national audit of practices, literature review and proposed management

Dhyna Thorel
Daniel Benaïm
  • Fonction : Auteur
Vincent Daien
  • Fonction : Auteur
Eric Gabison
  • Fonction : Auteur
Valentine Saunier
  • Fonction : Auteur
Laurence Béral
  • Fonction : Auteur
David Touboul
  • Fonction : Auteur
Dominique Brémond-Gignac
  • Fonction : Auteur
Matthieu Robert
  • Fonction : Auteur
Robin Vasseur
  • Fonction : Auteur
Gérard Royer
  • Fonction : Auteur
Brigitte Milpied
  • Fonction : Auteur
Claire Bernier
  • Fonction : Auteur
Anne Welfringer-Morin
  • Fonction : Auteur
Christine Bodemer
Nadège Cordel
Marie Tauber
  • Fonction : Auteur
Carole Burillon
  • Fonction : Auteur
Marion Servant
  • Fonction : Auteur
Chloe Couret
  • Fonction : Auteur
Bertrand Vabres
  • Fonction : Auteur
Florence Tétart
  • Fonction : Auteur
Myriam Cassagne
  • Fonction : Auteur
Marie-Ange Kuoch
  • Fonction : Auteur
Marc Muraine
  • Fonction : Auteur
Agnès Delcampe
  • Fonction : Auteur
Julie Gueudry
  • Fonction : Auteur

Résumé

Abstract Stevens-Johnson Syndrome (SJS) and toxic epidermal necrolysis (TEN) are serious and rare diseases, most often drug-induced, and their incidence has been estimated at 6 cases/million/year in France. SJS and TEN belong to the same spectrum of disease known as epidermal necrolysis (EN). They are characterized by more or less extensive epidermal detachment, associated with mucous membrane involvement, and may be complicated during the acute phase by fatal multiorgan failure. SJS and TEN can lead to severe ophthalmologic sequelae. There are no recommendations for ocular management during the chronic 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. Ophthalmologists and dermatologists from the French reference center for epidermal necrolysis were asked to complete a questionnaire on management practices in the chronic phase of SJS/TEN. The survey focused on the presence of a referent ophthalmologist at the center, the use of local treatments (artificial tears, corticosteroid eye drops, antibiotic-corticosteroids, antiseptics, vitamin A ointment (VA), cyclosporine, tacrolimus), the management of trichiatic eyelashes, meibomian dysfunction, symblepharons, and corneal neovascularization, as well as the contactologic solutions implemented. Eleven ophthalmologists and 9 dermatologists from 9 of the 11 centers responded to the questionnaire. Based on questionnaire results, 10/11 ophthalmologists systematically prescribed preservative-free artificial tears, and 11/11 administered VA. Antiseptic or antibiotic eye drops or antibiotic-corticosteroid eye drops were recommended as needed by 8/11 and 7/11 ophthalmologists, respectively. In case of chronic inflammation, topical cyclosporine was consistently proposed by 11/11 ophthalmologists. The removal of trichiatic eyelashes was mainly performed by 10/11 ophthalmologists. Patients were referred to a reference center for fitting of scleral lenses (10/10,100%). Based on this practice audit and literature review, we propose an evaluation form to facilitate ophthalmic data collection in the chronic phase of EN and we also propose an algorithm for the ophthalmologic management of ocular sequelae.

Domaines

Dermatologie
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Dates et versions

hal-04099619 , version 1 (30-10-2023)

Identifiants

Citer

Dhyna Thorel, Saskia Ingen-Housz-Oro, Daniel Benaïm, Vincent Daien, Eric Gabison, et al.. Ocular sequelae of epidermal necrolysis: French national audit of practices, literature review and proposed management. Orphanet Journal of Rare Diseases, 2023, 18 (1), pp.51. ⟨10.1186/s13023-023-02616-6⟩. ⟨hal-04099619⟩
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