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Chronic Ocular Sequelae of Stevens-Johnson Syndrome: Recent Advances in Understanding of Patho-Physiology and Management

Purvasha Narang,Sharon D’Souza

Published 2025 in Seminars in Ophthalmology

ABSTRACT

ABSTRACT Background Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe mucocutaneous reactions primarily triggered by drugs or infections. Although acute management focuses on systemic stabilization, chronic ocular sequelae remain a significant concern with vision-threatening complications arising in up to 75% of the affected patients. Ocular manifestations range from dry eye disease, lid margin keratinization (LMK), and conjunctival cicatrization to more severe complications like limbal stem cell deficiency (LSCD), cicatricial entropion, symblepharon, corneal perforation and scarring. Purpose This review aims to summarize the recent advances in understanding the pathophysiology and management strategies for chronic ocular sequelae of SJS. Methods A comprehensive literature search was conducted using PubMed database, identifying published studies addressing the roles of persistent immune dysregulation, autoantibodies, and microbiome alterations in perpetuating chronic ocular inflammation and scarring. The recent treatment modalities for such sequelae of SJS were also evaluated. Conclusion Elevated levels of pro-inflammatory cytokines, neutrophil predominance, and aberrant keratinization pathways have been identified as key contributors. Management strategies have evolved to include timely surgical interventions like mucous membrane grafting (MMG) for LMK, scleral lenses for ocular surface protection, and limbal stem cell transplantation for LSCD. The introduction of advanced therapies, such as topical autologous serum, platelet-rich plasma, and recombinant growth factors, has enhanced ocular surface rehabilitation. Emerging treatments, including simple oral mucosal epithelial transplantation (SOMET), cultivated oral mucosal epithelial transplantation (COMET), and keratoprostheses, offer hope for severe cases. Additionally, the integration of artificial intelligence (AI) in prognosis prediction and the development of targeted biologics highlight the potential for personalized care. Despite these advancements, challenges persist in early diagnosis, access to specialized care, and the high cost of novel therapies. Multidisciplinary collaboration and awareness are imperative for optimizing outcomes. Future directions emphasize the need for precision medicine approaches, AI integration, and nationwide registries to facilitate research and enhance patient support. Such comprehensive understanding and management approach aims to mitigate the long-term visual disability associated with chronic ocular sequelae of SJS/TEN, thereby improving quality of life.

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