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

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.

Topical treatment with a new matrix therapy agent (RGTA) for the treatment of corneal neurotrophic ulcers.

PURPOSE: Neurotrophic keratopathy is a degenerative disease of the corneal epithelium resulting from impaired corneal innervation, possibly leading to perforation. We aimed to assess the efficacy and tolerance of a new matrix therapy agent (RGTA, Cacicol20), mimicking heparan sulfates, for the management of neurotrophic keratopathy. METHODS: We carried out an uncontrolled, prospective, single-center clinical study on 11 patients (11 eyes) with severe corneal neurotrophic ulcers, despite the use of preservative-free artificial tears, for 15 days. Patients were treated with RGTA eye drops, instilled at a dosage of one drop in the morning, on alternate days. Evolution and follow-up during treatment were evaluated by slit-lamp examination, photography, fluorescein-dye testing, tests of corneal sensitivity, and best corrected visual acuity. The main outcome measures for each patient were healing of the corneal surface and best corrected visual acuity before and after RGTA therapy. RESULTS: Eight patients displayed complete corneal healing after a mean period of 8.7 weeks (range; 1 to 22 weeks). Mean ulcer area decreased significantly, from 11.12% to 6.37% (P = 0.048) in the first week, and to 1.56% (P = 0.005) at 1 month. Treatment failure was observed in three cases, requiring amniotic membrane transplantation in two patients and penetrating keratoplasty in one patient. At the end of the study, none of the patients displayed significant improvement in visual acuity. There were no systemic or local side effects of treatment. CONCLUSIONS: RGTA seems to be a potentially useful, alternative, noninvasive therapeutic approach in neurotrophic keratopathy management. However, randomized studies are necessary.

Vision-related function after scleral lens fitting in ocular complications of Stevens-Johnson syndrome and toxic epidermal necrolysis.

PURPOSE: To describe the therapeutic benefits of scleral lenses in the management of severe ocular surface disease attributable to toxic epidermal necrolysis (TEN) and Stevens-Johnson syndrome (SJS). DESIGN: Retrospective study. METHODS: Clinical records of 39 patients (67 eyes) fitted with scleral lenses for refractory ocular surface disease attributable to SJS or TEN were reviewed. To assess vision-specific quality of life, each patient completed the Ocular Surface Disease Index (OSDI) questionnaire and the National Eye Institute Visual Function Questionnaire (NEI VFQ-25). Slit-lamp examination was performed at regular intervals to detect side effects. Main outcome measures were best-corrected visual acuity (VA) and OSDI and NEI VFQ-25 composite score before and 6 months after scleral lens fitting. RESULTS: The mean age was 35.8 +/- 13.9 years. Scleral lens fitting failed in 3 patients. The mean follow-up was 33.3 +/- 17.6 months. Among fitted patients, VA in the better eye (36 patients, 36 eyes) progressed from 0.73 to 0.50 logarithm of the minimum angle of resolution (P = .0001) 6 months after scleral lens placement. The mean OSDI improved from 76.9 +/- 22.8 to 37.1 +/- 26.7 (P = .0001). Thirty-two NEI VFQ-25 composite scores were available. The mean NEI VFQ-25 composite score improved from 25.1 +/- 16.8 to 67.4 +/- 22.1 (P = .0001). No serious adverse events attributable to the scleral lenses occurred. CONCLUSIONS: Scleral lens use appears to be efficient and safe for visual rehabilitation of refractory ocular surface disease attributable to TEN and SJS.

In vivo confocal microscopic evaluation of corneal changes in chronic Stevens-Johnson syndrome and toxic epidermal necrolysis.

PURPOSE: To describe corneal changes visible on in vivo confocal microscopy, in patients with debilitating ocular sequelae because of toxic epidermal necrolysis (TEN) or Stevens-Johnson syndrome (SJS). PATIENTS AND METHODS: Forty-one eyes of 25 consecutive patients suffering from chronic TEN or SJS were studied using in vivo confocal microscopy. RESULTS: Severe dry eye syndrome with no associated limbal stem cell deficiency (25 eyes, 16 patients, 61%) was the most frequent clinical pattern. Limbal stem cell deficiency was noted in 16 eyes (12 patients, 39%). Three patients had asymmetric disease. Confocal microscopy showed a consistent change in the superficial epithelial cells in both clinical presentations. Patients with dry eye syndrome had frequent pathological nerve damages, and the presence of dendritic cells was prevalent (65%). Inflammatory cells were observed in a large number in 4 of the 12 patients presenting neovascularization of the cornea. CONCLUSIONS: The corneas of patients with chronic ocular sequelae linked to SJS and TEN present a number of abnormalities. In vivo confocal microscopy is a potentially useful tool for therapeutic indications and for follow-up of the debilitating chronic ocular problems associated with these diseases.