Stevens-Johnson syndrome with ocular involvement

Pertinent questions when taking the history

• New medicationsBuild a full medication timeline. SJS is a delayed type IV hypersensitivity reaction that typically develops 4 to 28 days after first exposure. Latency can be shorter on re-exposure and up to about 8 weeks with long half-life drugs such as allopurinol, phenytoin, carbamazepine, and lamotrigine.
• Time since onsetEarly treatment matters. Japanese data show fewer ocular sequelae when steroid pulse therapy and intensive topical steroids start within 4 days of onset.
• Cold medications, NSAIDs, or a common cold prodromeAssociated with severe ocular complications. Raise suspicion even if the presentation otherwise looks limited. Genetic susceptibility involving TLR3, EP3, and HLA alleles has been reported, with differences between populations.
• Ocular symptomsCan be masked by systemic illness. Keep a high suspicion for ophthalmic involvement and monitor with visual vital signs and a fluorescein exam.
• Pre-existing dry eyeMay call for more aggressive therapy.
• Before topical steroidsScreen for glaucoma or raised pressure, which call for closer monitoring, and for active ocular infection, including HSV epithelial keratitis, which changes the treatment plan.
• DemographicsFindings are inconsistent. A large international cohort found severe ocular complications more often in patients under 30 and in women; a smaller acute cohort found older age marginally predictive.

Visual vital signs

Vision, pressure, and pupils, recorded and handed off like any other vital sign.

Describe the focused anatomy

• External examDo the periorbital tissues show edema, erythema, scaling, or sloughing? On one side or both? Does it reach the lid margin and spread onto the palpebral conjunctiva, or are the eyelids spared?
• Lid margins and mucocutaneous junctionEarly ocular involvement often shows erythema, sloughing, and heavy mucus production.
• Palpebral and bulbar conjunctiva and fornicesThe palpebral conjunctiva lines the inside of the eyelid; the bulbar conjunctiva covers the white of the eye. Look for injection of both and heavy mucus discharge. Flipping the lid with a cotton tip may show an opaque or grayish membrane (pseudomembrane). Pseudomembranes that are not removed or controlled with topical steroids can scar into symblepharon, with long term dry eye, poor tear film, trichiasis, distichiasis, and lid malposition. Results are best when therapy, including membrane removal and topical steroid and antibiotic where appropriate, starts early, before scar tissue forms.
• Cornea and limbusUse fluorescein to look for punctate epithelial erosions or epithelial defects. The cornea may look hazy with epithelial or stromal disease. An infiltrate, thinning, or suppuration raises concern for microbial keratitis. Progressive involvement can lead to limbal stem cell deficiency with conjunctivalization or keratinization, which can be permanent without treatment.
• Lacrimal and meibomian glandsInvolvement here dominates the chronic phase and calls for lubrication throughout treatment.

Supporting imaging, if needed

• None neededSJS is primarily a clinical diagnosis. Light sensitivity, foreign body sensation, or increasing discharge raise suspicion for eye involvement.
• Slit lamp and fluoresceinCharacterize the extent of epithelial involvement, which sets the staging, the frequency of topical therapy, and the follow up.

Treatment

• LubricationPreservative free artificial tears every 1 to 2 hours during the day, with lubricating ointment at night, regardless of severity.Paulmann 2024, German S3 guideline, recommendation 32; Metcalfe 2021.
• Topical steroidAn ophthalmology decision, ideally made at the first exam. Protocols commonly use prednisolone acetate 1%, with the pressure checked and monitored for a steroid response. Glaucoma calls for closer monitoring rather than automatic withholding. Suspected active infection, including HSV epithelial keratitis, changes the plan. If anti-inflammatory therapy is needed beyond 2 to 4 weeks, the German guideline suggests switching to a calcineurin inhibitor where possible.Paulmann 2024, recommendation 32; Shanbhag 2019 protocol.
• Topical antibioticWhen there is an epithelial defect, a preservative free fluoroquinolone such as ofloxacin or moxifloxacin. Practice varies without a defect: the German guideline advises against prophylaxis, while the Mass Eye and Ear protocol uses moxifloxacin routinely.Paulmann 2024, recommendation 33; Metcalfe 2021; Thorel 2020.
• Systemic steroidsOral or IV steroids may be needed in severe cases. Consider co-management with dermatology and other subspecialty teams.
• Amniotic membraneSevere lid margin, conjunctival, or corneal epithelial involvement calls for early amniotic membrane, decided by severity rather than after drops fail. Controlling the active inflammation helps avoid permanent cicatricial change, and membrane is far more effective in the acute phase than in later reconstruction.Gregory 2016; Shanbhag 2019; Paulmann 2024, recommendations 36 and 37.
• Autologous serum tearsMay be considered for severe surface damage or persistent epithelial defects, mainly after the acute phase. Concentrations and dosing vary between protocols.Paulmann 2024.
• TransferTransfer to an ICU or burn unit with ophthalmology services when the airway is at risk or extensive systemic involvement may cause hemodynamic instability.

Follow up

• While inpatientDaily ophthalmic examination through the first week when the lid margins, conjunctiva, or cornea are involved, and at least every 48 hours for every patient, since ocular findings can progress independently of the skin. Keep asking about symptoms and monitor with visual vital signs.Shanbhag 2019; Metcalfe 2021; Paulmann 2024.
• At each visitRe-grade fluorescein staining of the lid margins, conjunctiva, and cornea, pseudomembranes, and visual acuity. Consider initial or repeat amniotic membrane as severity changes, and titrate acute medical therapy to the systemic course.
• After dischargeIndividualized to severity. One well studied protocol used monthly visits for 3 months, every 4 to 6 months for 3 years, then at least yearly.Shanbhag 2019.
• PrioritiesHalt the acute inflammation with steroids, avoid or treat concurrent infection, remove pseudomembranes, preserve functioning conjunctival goblet cells, and avoid permanent cicatricial change.