**Background**
Stevens-Johnson Syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe, rare, drug-induced diseases with an incidence of 6 cases/million/year in France. They are characterized by epidermal and mucosal necrosis, and 20-79% of acute-phase patients develop vision-threatening ocular damage. Chronic ocular sequelae, including severe dry eye, limbal stem cell deficiency, and corneal opacification, occur in 35-50% of survivors and can lead to blindness. Despite this, no consensus exists for ophthalmologic management in the chronic phase. This study aimed to audit current practices across the French reference center for toxic bullous dermatoses (TOXIBUL), review the literature, and propose standardized guidelines.
**Methods**
A standardized questionnaire was emailed to ophthalmologists and dermatologists at all 11 TOXIBUL centers. The survey covered the presence of a referent ophthalmologist, use of topical treatments (artificial tears, corticosteroids, antibiotics, vitamin A ointment, cyclosporine, tacrolimus, autologous serum), management of trichiasis, meibomian gland dysfunction, symblepharons, corneal neovascularization, and contactologic solutions. A literature review was conducted via PubMed for articles published between 1987 and 2021 on chronic-phase management of SJS/TEN, focusing on topical treatments, systemic immunosuppressants, eyelid treatments, and adjuvant therapies (amniotic membrane transplantation, scleral lenses, anti-VEGF). Single case reports and acute-phase articles were excluded. Results from the audit and literature were synthesized into a proposed evaluation form and management algorithm.
**Key Results**
Eleven ophthalmologists and 9 dermatologists from 9 of 11 centers responded. All dermatologists (9/9) reported having a referent ophthalmologist. Among ophthalmologists, 10/11 (91%) systematically prescribed preservative-free artificial tears, and 11/11 (100%) used vitamin A ointment. Antiseptic or antibiotic eye drops were used as needed by 8/11 (73%), and antibiotic-corticosteroid drops by 7/11 (64%). For chronic inflammation, topical cyclosporine was consistently proposed by 11/11 (100%), while tacrolimus was used by 4/11 (36%) and autologous serum by 6/11 (55%). Systemic immunosuppressants were never used (0/11). Trichiasis removal was performed by the ophthalmologist in 10/11 (91%) cases, with argon laser used by 7/11 (64%). Symblepharon release was not systematic (8/10, 80%) and was reserved for difficulty with scleral lens fitting (8/10, 80%) or severe eyelid malposition (9/10, 90%), often combined with amniotic membrane or oral mucosa transplant (7/10, 70%). For corneal ulcers, amniotic membrane transplant was recommended by 9/11 (82%) and tectonic keratoplasty by 6/11 (55%). All ophthalmologists (11/11, 100%) recommended eyelid hygiene for meibomian gland dysfunction, with topical antibiotics as needed (10/11, 91%). Scleral lenses were offered by all centers for disabling keratoconjunctivitis sicca (10/10, 100%), and anti-VEGF was used for corneal neovascularization by 7/11 (64%).
The literature review identified 39 articles, with 19 summarized. Key findings included: preservative-free artificial tears are essential; vitamin A ointment reduces conjunctival keratinization; short-term corticosteroids are anti-inflammatory but long-term use is not recommended due to side effects; cyclosporine 0.05% eye drops improve dry eye symptoms (p<0.05); autologous serum eye drops contain growth factors but are limited by production requirements; topical tacrolimus has a corticosteroid-sparing effect (p=0.004); systemic immunosuppressants are reserved for perioperative or refractory inflammation; oral mucosa transplant for entropion shows 83% complete resolution; subconjunctival anti-VEGF reduces corneal neovascularization (p=0.0003) but does not improve visual acuity; scleral lenses improve visual acuity (p=0.0001) and quality of life; and limbal stem cell transplant improves visual acuity in 82% of eyes at 2 years.
**Clinical Implications**
This study demonstrates a relatively uniform approach to managing chronic ocular sequelae of SJS/TEN across French reference centers, despite the absence of formal guidelines. The proposed evaluation form and algorithm provide a structured framework for clinicians, emphasizing stepwise management: first addressing ocular surface inflammation and mechanical factors (e.g., trichiasis, symblepharons), then pursuing visual rehabilitation with scleral lenses or advanced surgical options like limbal stem cell transplant or osteodontokeratoprosthesis. The findings highlight the importance of multidisciplinary care, long-term follow-up, and referral to specialized centers for complex interventions. These recommendations aim to standardize care, improve patient outcomes, and reduce the risk of blindness in this rare but devastating condition.