Ophthalmic Aspects of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: A Narrative Review

Abstract

The aim of our review article was to summarize the current literature on Stevens-Johnson syndrome (SJS) and its severe form, toxic epidermal necrolysis (TEN). SJS/TEN is a serious, rare multi-system, immune-mediated, mucocutaneous disease with a significant mortality rate that can lead to severe ocular surface sequelae and even to bilateral blindness. Restoration of the ocular surface in acute and chronic SJS/TEN is challenging. There are only limited local or systemic treatment options for SJS/TEN. Early diagnosis, timely amniotic membrane transplantation and aggressive topical management in acute SJS/TEN are necessary to prevent long-term, chronic ocular complications. Although the primary aim of acute care is to save the life of the patient, ophthalmologists should regularly examine patients already in the acute phase, which should also be followed by systematic ophthalmic examination in the chronic phase. Herein, we summarize actual knowledge on the epidemiology, aetiology, pathology, clinical appearance and treatment of SJS/TEN.

Keywords: Corneal blindness; Stevens–Johnson syndrome; Toxic epidermal necrolysis.

Fig. 1

Ocular signs of acute Stevens–Johnson syndrome/toxic epidermal necrolysis. A BA: widespread eyelid margin necrosis, WA: conjunctival chemosis; B BA: pseudomembrane development, WA: conjunctival hyperaemia. BA black arrow, WA  white arrow

Fig. 2

Chronic ocular sequelae of Stevens–Johnson syndrome/toxic epidermal necrolysis. A BA: trichiasis; B BA: symblepharon, WA: corneal keratinization; C BA: conjunctivalization of the cornea, WA: corneal neovascularization, S: symblepharon; D BA: corneal neovascularization, WA: corneal keratinization; E BA: meibomian gland destruction, WA: lid margin keratinization; F BA: symblepharon, WA: meibomian gland destruction, S: corneal keratinization. BA  black arrow, WA  white arrow, S  star