Lid-Related Keratopathy in Stevens-Johnson Syndrome: Natural Course and Impact of Therapeutic Interventions in Children and Adults

Our purpose is to comprehensively review the state of the art with regard to Stevens- Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN), with particular attention to improving the management of associated ocular surface complications. SJS and TEN are two ends of a spectrum of immune-mediated disease, characterized in the acute phase by a febrile illness followed by skin and mucous membrane necrosis and detachment. Part I of this review focused on the systemic aspects of SJS/TEN and was published in the January 2016 issue of this journal. The purpose of Part II is to summarize the ocular manifestations and their management through all phases of SJS/TEN, from acute to chronic. We hope this effort will assist ophthalmologists in their management of SJS/TEN, so that patients with this complex and debilitating disease receive the best possible care and experience the most optimal outcomes in their vision and quality of life.

To evaluate and grade the extent and severity of chronic ocular manifestations in Stevens-Johnson syndrome (SJS). Prospective multicenter case series. We enrolled 73 patients (138 eyes) with SJS seen between April 2003 and March 2005 at 3 tertiary referral centers. Patients with a confirmed history of SJS and chronic ocular complications that persisted for at least 1 year from the onset of SJS were included. Their detailed medical history and ophthalmic examination results were recorded on an itemized data collection form. Complications were categorized as corneal, conjunctival, and eyelid complications, and 13 components were evaluated and graded on a scale from 0 to 3 according to their severity. These were broadly classified as corneal (superficial punctate keratopathy, epithelial defect, loss of the palisades of Vogt, conjunctivalization, neovascularization, opacification, keratinization), conjunctival (hyperemia, symblepharon formation), and eyelid (trichiasis, mucocutaneous junction involvement, meibomian gland involvement, punctal damage) complications. The most severely affected complication components were loss of the palisades of Vogt (114 eyes; 82.6%) and meibomian gland involvement (102 eyes; 73.9%). Visual acuity in 74 of the 138 eyes (53.6%) was worse than 20/200. The severity of corneal, conjunctival, and eyelid complications was significantly correlated with visual loss. All 13 complications were correlated significantly with logarithm of the minimum angle of resolution (logMAR) visual acuity; the correlation coefficient (R) ranged from 0.359 to 0.810 (P<0.0001); for corneal epithelial defects, R was 0.169 (P = 0.0473). Eyes with a higher total score for the 3 complication categories had poorer vision (R = 0.806; P<0.0001). Multivariate regression analysis showed that corneal neovascularization, opacification, keratinization, and cataracts significantly affected logMAR visual acuity (P<0.0001, P<0.0001, P = 0.0142, P = 0.0375, respectively). The authors describe a new method for grading the extent and severity of ocular involvement in patients with SJS and demonstrate that the severity of ocular involvement is correlated significantly with the final visual outcome. This new grading system provides a more objective method for evaluating SJS patients and may be adapted for use in other cicatricial ocular surface diseases.

To describe the acute and late ocular manifestations and complications in toxic epidermal necrosis (TEN) and Stevens-Johnson syndrome (SJS), and identify predictors for development of late complications. Cases of TEN and SJS during a 9-year period were included. Patients with ocular involvement were reviewed for acute ocular complications. Patients with a minimum 6 months follow-up were reviewed for late complications. Records were reviewed for their demographics, etiology, and severity of ocular involvement. There were 117 patients with a mean age of 52.2 +/- 18.6 years. Eighty-one of these (69%) had acute ocular involvement. This was mild in 40%, moderate in 25% and severe in 4%. Adverse drug reactions were the predominant cause. Patients with thrombocytopenia had more severe acute ocular involvement. Forty-four patients had a minimum 6 months of follow-up and half developed late complications. Severe dry eyes and trichiatic lashes were the commonest late complications. Patients treated with topical antibiotic were more likely to have late complications, particularly dry eyes. There was no difference in the severity of acute eye involvement or late complications when SJS and TEN patients were compared. The severity of the acute ocular disease and abnormal laboratory tests were not found to be the significant risk factors of late complications. Ocular involvement is common in SJS and TEN and can be severe and blinding. The severity of acute ocular complications does not predict late complications. The diagnosis of TEN does not imply a more severe ocular involvement or increased frequency of late ocular complications compared with SJS. Care should be taken even in mild cases. Appropriate intervention during acute ocular disease may prevent late complications.