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 ,N Bellon ,M Tauber ,T Cornut ,C Couret ,M Muraine ,François Malecaze ,B Vabres ,Matthieu P Robert ,N Cordel ,G Royer ,B Milpied ,Christine Bodemer ,A Welfringer‐Morin ,L Beral ,P Wolkenstein ,F Tétart ,O Dereure ,C Bernier ,Nicolas De Prost ,B Ben Saïd ,Vincent Daïen ,Saskia Ingen‐Housz‐Oro ,Dominique Brémond‐Gignac ,A Colin ,A Delcampe ,C Burillon ,J Gueudry

doi:10.1186/s13023-020-01538-x

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.

Highlights

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN, or Lyell syndrome) are serious and rare diseases, most often drug-induced, and their incidence has recently been re-estimated at 6 cases/million/year in France [1]
The bibliographic search was guided by three themes: type of local drug administration, type of systemic treatment (i.e. systemic steroid therapy, intravenous immunoglobulin (IVIg), and cyclosporine), and type of adjuvant treatment and/or ophthalmologic surgery such as amniotic membrane transplantation (AMT)
Our national audit of medical practices in France reveals that ophthalmologic management during the acute phase of SJS/TEN relies mainly on early evaluation by an ophthalmologist, surgical debridement of pseudomembranes, and application of artificial tears and/or vitamin A eye ointment

Summary

Introduction

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN, or Lyell syndrome) are serious and rare diseases, most often drug-induced, and their incidence has recently been re-estimated at 6 cases/million/year in France [1]. SJS and TEN are characterized by widespread necrosis of the epidermis and the mucous membrane, sometimes complicated during the acute phase by multiorgan failure that can be fatal. They differ according to the extent of skin detachment they cause, SJS < 10%, TEN or Lyell syndrome ≥30%, and SJS/TEN overlap 10– 29% [2]. The severe degree consists in the formation of symblepharon, persistent non-healing keratitis despite medical treatment, and/or decrease in visual acuity, and/or conjunctival fornix foreshortening. Another classification has been proposed by Sotozono et al [5]. Skin phototypes V and VI have recently been reported as an additional risk factor for more severe, long-term ocular complications [11]

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Results

Conclusion