Ocular sequelae of epidermal necrolysis: French national audit of practices, literature review and proposed management
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 a...
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| Format: | Article |
| Language: | English |
| Published: |
BMC
2023-03-01
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| Series: | Orphanet Journal of Rare Diseases |
| Subjects: | |
| Online Access: | https://doi.org/10.1186/s13023-023-02616-6 |
| _version_ | 1797863531439194112 |
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| author | Dhyna Thorel Saskia Ingen-Housz-Oro Daniel Benaïm Vincent Daien Eric Gabison Valentine Saunier Laurence Béral David Touboul Dominique Brémond-Gignac Matthieu Robert Robin Vasseur Gérard Royer Olivier Dereure Brigitte Milpied Claire Bernier Anne Welfringer-Morin Christine Bodemer Nadège Cordel Marie Tauber Carole Burillon Marion Servant Chloe Couret Bertrand Vabres Florence Tétart Myriam Cassagne Marie-Ange Kuoch Marc Muraine Agnès Delcampe Julie Gueudry |
| author_facet | Dhyna Thorel Saskia Ingen-Housz-Oro Daniel Benaïm Vincent Daien Eric Gabison Valentine Saunier Laurence Béral David Touboul Dominique Brémond-Gignac Matthieu Robert Robin Vasseur Gérard Royer Olivier Dereure Brigitte Milpied Claire Bernier Anne Welfringer-Morin Christine Bodemer Nadège Cordel Marie Tauber Carole Burillon Marion Servant Chloe Couret Bertrand Vabres Florence Tétart Myriam Cassagne Marie-Ange Kuoch Marc Muraine Agnès Delcampe Julie Gueudry |
| author_sort | Dhyna Thorel |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-04-09T22:38:04Z |
| format | Article |
| id | doaj.art-4e2db3aecc9e4874ab95d0b65729eb2b |
| institution | Directory Open Access Journal |
| issn | 1750-1172 |
| language | English |
| last_indexed | 2024-04-09T22:38:04Z |
| publishDate | 2023-03-01 |
| publisher | BMC |
| record_format | Article |
| series | Orphanet Journal of Rare Diseases |
| spelling | doaj.art-4e2db3aecc9e4874ab95d0b65729eb2b2023-03-22T12:21:59ZengBMCOrphanet Journal of Rare Diseases1750-11722023-03-0118111410.1186/s13023-023-02616-6Ocular sequelae of epidermal necrolysis: French national audit of practices, literature review and proposed managementDhyna Thorel0Saskia Ingen-Housz-Oro1Daniel Benaïm2Vincent Daien3Eric Gabison4Valentine Saunier5Laurence Béral6David Touboul7Dominique Brémond-Gignac8Matthieu Robert9Robin Vasseur10Gérard Royer11Olivier Dereure12Brigitte Milpied13Claire Bernier14Anne Welfringer-Morin15Christine Bodemer16Nadège Cordel17Marie Tauber18Carole Burillon19Marion Servant20Chloe Couret21Bertrand Vabres22Florence Tétart23Myriam Cassagne24Marie-Ange Kuoch25Marc Muraine26Agnès Delcampe27Julie Gueudry28Ophthalmology Department, CHU Charles Nicolle RouenReference Center for Toxic Bullous Dermatoses and Severe Drug Reactions TOXIBULReference Center for Toxic Bullous Dermatoses and Severe Drug Reactions TOXIBULReference Center for Toxic Bullous Dermatoses and Severe Drug Reactions TOXIBUL6Ophthalmology Department, Hôpital Fondation Adolphe de RothschildReference Center for Toxic Bullous Dermatoses and Severe Drug Reactions TOXIBULReference Center for Toxic Bullous Dermatoses and Severe Drug Reactions TOXIBULReference Center for Toxic Bullous Dermatoses and Severe Drug Reactions TOXIBULReference Center for Toxic Bullous Dermatoses and Severe Drug Reactions TOXIBULReference Center for Toxic Bullous Dermatoses and Severe Drug Reactions TOXIBULReference Center for Toxic Bullous Dermatoses and Severe Drug Reactions TOXIBULReference Center for Toxic Bullous Dermatoses and Severe Drug Reactions TOXIBULReference Center for Toxic Bullous Dermatoses and Severe Drug Reactions TOXIBULReference Center for Toxic Bullous Dermatoses and Severe Drug Reactions TOXIBULReference Center for Toxic Bullous Dermatoses and Severe Drug Reactions TOXIBULReference Center for Toxic Bullous Dermatoses and Severe Drug Reactions TOXIBULReference Center for Toxic Bullous Dermatoses and Severe Drug Reactions TOXIBULReference Center for Toxic Bullous Dermatoses and Severe Drug Reactions TOXIBULReference Center for Toxic Bullous Dermatoses and Severe Drug Reactions TOXIBULReference Center for Toxic Bullous Dermatoses and Severe Drug Reactions TOXIBULReference Center for Toxic Bullous Dermatoses and Severe Drug Reactions TOXIBULReference Center for Toxic Bullous Dermatoses and Severe Drug Reactions TOXIBULReference Center for Toxic Bullous Dermatoses and Severe Drug Reactions TOXIBULOphthalmology Department, CHU Charles Nicolle RouenReference Center for Toxic Bullous Dermatoses and Severe Drug Reactions TOXIBULReference Center for Toxic Bullous Dermatoses and Severe Drug Reactions TOXIBULOphthalmology Department, CHU Charles Nicolle RouenOphthalmology Department, CHU Charles Nicolle RouenOphthalmology Department, CHU Charles Nicolle RouenAbstract 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.https://doi.org/10.1186/s13023-023-02616-6Stevens-Johnson syndromeToxic epidermal necrolysisManagementOcular involvementTreatmentDrug reaction |
| spellingShingle | Dhyna Thorel Saskia Ingen-Housz-Oro Daniel Benaïm Vincent Daien Eric Gabison Valentine Saunier Laurence Béral David Touboul Dominique Brémond-Gignac Matthieu Robert Robin Vasseur Gérard Royer Olivier Dereure Brigitte Milpied Claire Bernier Anne Welfringer-Morin Christine Bodemer Nadège Cordel Marie Tauber Carole Burillon Marion Servant Chloe Couret Bertrand Vabres Florence Tétart Myriam Cassagne Marie-Ange Kuoch Marc Muraine Agnès Delcampe Julie Gueudry Ocular sequelae of epidermal necrolysis: French national audit of practices, literature review and proposed management Orphanet Journal of Rare Diseases Stevens-Johnson syndrome Toxic epidermal necrolysis Management Ocular involvement Treatment Drug reaction |
| title | Ocular sequelae of epidermal necrolysis: French national audit of practices, literature review and proposed management |
| title_full | Ocular sequelae of epidermal necrolysis: French national audit of practices, literature review and proposed management |
| title_fullStr | Ocular sequelae of epidermal necrolysis: French national audit of practices, literature review and proposed management |
| title_full_unstemmed | Ocular sequelae of epidermal necrolysis: French national audit of practices, literature review and proposed management |
| title_short | Ocular sequelae of epidermal necrolysis: French national audit of practices, literature review and proposed management |
| title_sort | ocular sequelae of epidermal necrolysis french national audit of practices literature review and proposed management |
| topic | Stevens-Johnson syndrome Toxic epidermal necrolysis Management Ocular involvement Treatment Drug reaction |
| url | https://doi.org/10.1186/s13023-023-02616-6 |
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