スティーヴンス・ジョンソン症候群

PURPOSE: To evaluate the long-term visual and therapeutic outcomes of scleral lenses in pediatric patients with ocular surface disorders over a 17-year period at a tertiary eye care center. METHODS: This retrospective observational study included pediatric patients (≤18 years) with ocular surface disorders who underwent scleral lens fitting between 2008 and 2025 at a tertiary center in South India, with a minimum follow-up of 12 months. Data extracted from electronic medical records included demographics, diagnosis, best-corrected visual acuity (BCVA), corneal neovascularization, surface keratinization, lubricant dependence, prior surgeries, and lens-related complications. Corneal neovascularization and keratinization were graded on a 0 to 3 scale. Snellen BCVA was converted to logMAR for analysis. Primary outcomes were change in visual acuity and ocular surface parameters. RESULTS: Ninety-one eyes of 67 pediatric patients (45 males, 22 females) were analyzed. Mean age at lens insertion was 10.63 ± 3.23 years (range 4-17), with mean follow-up of 40.0 ± 22.32 months. Among eyes with chemical injury, 10/14 had prior simple limbal epithelial transplantation, whereas 22/75 eyes with Stevens-Johnson syndrome underwent symblepharon release before fitting. BCVA improved significantly (P < 0.05) after lens fitting. Corneal neovascularization decreased in 73% of eyes, lubricant dependence reduced in 41.8%, and 66.7% of SJS eyes showed reduced keratinization. Complications included conjunctival impingement (12%) and lens breakage (2.2%); no lens-related infections occurred. CONCLUSIONS: Scleral lenses provide safe, sustained visual improvement and ocular surface stabilization in pediatric ocular surface disease and should be considered an integral component of multidisciplinary care.

PURPOSE: To describe the management of fast mucosal overgrowth by trimming it and application of a low concentration of mitomycin C (MMC) in a Boston Keratoprosthesis type I with buccal autologous mucosa overlay (B1KProM). METHODS: We report a single-patient case in which low-dose, short-duration MMC was used. A review of the literature was performed to identify previously reported dosing strategies. A 21-year-old woman developed ibuprofen-induced Stevens-Johnson syndrome at age 5, resulting in severe ocular surface disease. Multiple surgeries performed on the right eye (RE) failed because of persistent epithelial defects and recurrent infections. The best-corrected visual acuity was 0.08 (decimal) in the RE. A B1KProM was implanted on the RE but after 2 weeks mucosa overgrown, partially obstructing the visual axis. A resection of the mucosal excess plus application of a sponge soaked in MMC 0.01% for 1 minute was performed to prevent recurrence. RESULTS: The intervention successfully restored the visual axis preserving buccal mucosa integration during the 12 months of follow-up. In this period, no MMC complications were found and the best-corrected visual acuity improved to 0.6. The prosthesis remained in place without additional postoperative complications. CONCLUSIONS: Mucosal overgrowth is a frequent complication after B1KProM, particularly in eyes with Stevens-Johnson syndrome. This report provides initial evidence that the complication could be successfully treated with mucosal excision and a lower dose of MMC, to prevent recurrence, avoiding side effects, and to improve prosthesis retention, usually shorter in patients with autoimmune disease.

BACKGROUND: Allopurinol-induced severe cutaneous adverse reactions (SCARs) are rare but potentially life threatening, particularly in Asian populations. While the genetic marker HLA-B*58:01 is a well-established risk factor, non-genetic factors may also contribute. This systematic review synthesizes evidence on associations between non-genetic risk factors and allopurinol-induced SCAR. METHODS: We searched MEDLINE, Scopus, Cochrane Library and Web of Science from inception to 29 June 2026 for observational studies examining non-genetic risk factors for SCAR, defined as Stevens-Johnson Syndrome, Toxic Epidermal Necrolysis, Acute Generalised Exanthematous Pustulosis, or Hypersensitivity Syndrome/Drug Reaction with Eosinophilia and Systemic Symptoms. Adults aged ≥ 18 years were included. Pooled odds ratios (ORs) with 95% confidence intervals (CIs) were calculated using a random-effects model; heterogeneity was assessed with I2. Mean differences were calculated for continuous variables. NIH Study Quality Assessment Tool was used for quality assessment of the studies. RESULTS: Twenty-six studies were included. Female sex (20 studies; 3340 SCAR cases, 562,647 controls) was associated with an increased risk of allopurinol-induced SCAR (OR 2.06; 95% confidence interval (CI) 1.25-3.38). Chronic kidney disease (16 studies; 1625 SCAR cases, 553,804 controls) was also significantly associated with SCAR (OR 3.78; 95% CI 1.99-7.17). Five studies (177 SCAR cases, 1368 controls) reported higher allopurinol doses among SCAR cases than tolerant controls (mean difference 19.61 mg; 95% CI 2.97-36.24). No significant associations were observed for age or concomitant diuretic use in the primary meta-analyses. Substantial heterogeneity was observed across studies. Sensitivity analyses demonstrated consistent findings for most factors, although concomitant diuretic use became significantly associated with SCAR after exclusion of non-Asian and zero-event studies. CONCLUSION: CKD, female sex, and higher allopurinol dose were identified as significant non-genetic risk factors for allopurinol-induced SCAR. These findings support consideration of non-genetic factors alongside pharmacogenomic screening in future risk-stratification strategies. However, substantial heterogeneity and potential publication bias limit the certainty of the available evidence. Well-designed studies evaluating non-genetic predictors as primary outcomes are needed to develop robust integrated risk prediction models for clinical decision making.

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are acute, life-threatening mucocutaneous reactions causing necrosis of external and internal body surfaces, thereby predisposing patients to multiorgan complications. Numerous internal organ systems are impacted by SJS-TEN, encompassing the skin, ocular, ear, nose, and throat (ENT), pulmonary, gastrointestinal (GIT), and genitourinary systems. The acute and long-term organ complications associated with SJS-TEN have not been comprehensively documented. The objective of this review is to systematically delineate multiorgan complications in cases of SJS-TEN. A comprehensive search of the available literature was conducted on electronic databases (PubMed and Scopus) published between 2019 and 2024, reporting SJS-TEN patients with multiorgan complications using predefined search terms and inclusion/exclusion criteria. The terms "Stevens-Johnson syndrome", "toxic epidermal necrolysis", "organ complication", "multiorgan involvement", and "sequelae" were used as keywords. We included 38 articles reporting SJS-TEN patients with multiorgan complications, comprising 15 reviews, 11 original studies, 9 case reports, and 3 case series; data were synthesized descriptively, and no quantitative meta-analysis was performed. Reported percentages therefore reflect the proportion of included studies reporting a given complication, not patient-level prevalence. Among studies providing extractable patient-level data, the mean age of the subject population was 36.72 ± 26.58 years, with female predominance (88.2%). Ocular involvement was the complication most frequently reported across the included studies (65.8% of studies), followed by pulmonary and genitourinary involvement (26.32% of studies each) and skin complications (21.05% of studies). The most frequently reported ocular complications were trichiasis, distichiasis, and symblepharon, each noted in 60% of studies reporting ocular involvement. SJS-TEN represents a severe delayed-type hypersensitivity reaction with a high risk of long-term mucocutaneous disabling effects. Given the heterogeneity of the included study designs, these findings should be interpreted as an indication of how frequently each complication has been reported in the literature rather than as an estimate of true patient-level risk. This systematic review emphasizes the importance of clinician awareness to ensure early detection of multiorgan complications associated with SJS-TEN, thereby ensuring optimal, multidisciplinary patient management.