What is the primary treatment for a patient with Stevens-Johnson Syndrome (SJS)?

Treatment of Stevens-Johnson Syndrome

Immediately discontinue all suspected culprit medications and transfer the patient to a specialized burn unit or ICU with multidisciplinary expertise, particularly when body surface area involvement exceeds 10%, while initiating comprehensive supportive care as the cornerstone of management. 1, 2

Immediate Life-Saving Actions

• Stop all potential causative drugs immediately upon suspicion of SJS—this is the single most critical intervention that directly impacts survival 1, 2, 3
• Calculate SCORTEN within the first 24 hours to predict mortality risk (scores range from 0-7, with mortality ranging from 1% to 99%) 2, 4, 3
• Transfer without delay to centers with burn unit or ICU capabilities and multidisciplinary teams including dermatology, intensive care, ophthalmology, and specialist skincare nursing 1, 2, 4
• Barrier-nurse patients in a temperature-controlled room (25-28°C) on a pressure-relieving mattress with humidity control to prevent hypothermia and reduce infection risk 2, 4

Common Pitfall: Delayed transfer to specialized care significantly increases mortality risk—transfer should occur within hours of diagnosis, not days 2, 3

Supportive Care Framework

Fluid and Hemodynamic Management

• Provide careful fluid resuscitation to prevent end-organ hypoperfusion while avoiding overaggressive replacement that leads to pulmonary, cutaneous, and intestinal edema 1, 2, 4
• Monitor fluid balance with regular assessment of vital signs, urine output, and electrolytes 1, 2
• Use urinary catheterization when urogenital involvement causes dysuria or retention, or to monitor output 1, 2

Wound Care Protocol

• Handle skin with extreme care to minimize shearing forces that cause further epidermal detachment 1, 2, 4
• Leave detached epidermis in situ to act as a biological dressing 1, 2, 4
• Irrigate wounds gently using warmed sterile water, saline, or chlorhexidine (1/5000) 2
• Apply greasy emollient (50% white soft paraffin with 50% liquid paraffin) over the entire epidermis including denuded areas every few hours 2, 4
• Apply nonadherent dressings to denuded dermis with secondary foam or burn dressings to collect exudate 1, 2, 4
• Consider silver-containing products/dressings for sloughy areas only 2

Infection Prevention and Management

• Do not use prophylactic systemic antibiotics—indiscriminate administration increases skin colonization with resistant organisms, particularly Candida albicans 1, 2, 4, 5
• Take swabs for bacterial and candidal culture from three lesional areas on alternate days 2
• Monitor for rising C-reactive protein and neutrophilia as indicators of sepsis 2
• Administer targeted antimicrobial therapy only when clinical signs of infection appear (fever, hemodynamic instability, positive cultures) 1, 2, 5

Common Pitfall: Fever from SJS/TEN itself complicates detection of secondary sepsis—rely on multiple clinical parameters, not fever alone 2

Mucosal Management

Ocular Care (Critical for Preventing Permanent Vision Loss)

• Arrange ophthalmological examination within 24 hours of diagnosis with daily reviews throughout the acute illness 1, 2, 4, 3
• Apply preservative-free lubricant eye drops every 2 hours throughout the acute illness 1, 2, 4
• Perform daily ocular hygiene by an ophthalmologist or ophthalmically trained nurse to remove inflammatory debris and break down conjunctival adhesions 1, 2
• Administer broad-spectrum topical antibiotics when corneal fluorescein staining or ulceration is present 1, 4
• Consider topical corticosteroid drops under ophthalmologist supervision to reduce ocular surface damage 1
• Consider amniotic membrane transplantation (AMT) in the acute phase for significantly better visual outcomes compared to medical management alone 2

Common Pitfall: Failure to involve ophthalmology within 24 hours leads to permanent visual impairment—63% of patients with acute ocular involvement develop chronic complications 1, 3

Oral Care

• Perform daily oral review during the acute illness 2, 4
• Apply white soft paraffin ointment to the lips every 2 hours throughout the acute illness 2, 4
• Use anti-inflammatory oral rinse containing benzydamine hydrochloride every 3 hours, particularly before eating 1, 2, 4
• Use antiseptic oral rinse containing chlorhexidine twice daily 2, 4
• Apply topical anesthetics such as viscous lidocaine 2% or cocaine mouthwashes 2-5% for severe oral discomfort 1
• Treat candidal infection with nystatin oral suspension 100,000 units four times daily for 1 week, or miconazole 2

Urogenital Care

• Perform daily urogenital review during the acute illness 4
• Apply white soft paraffin ointment to urogenital skin and mucosae every 4 hours 1, 4
• Apply potent topical corticosteroid ointment once daily to involved, noneroded surfaces 4
• Apply silicone dressings (e.g., Mepitel) to eroded areas 4
• Consider vaginal dilators or tampons wrapped in Mepitel to prevent vaginal synechiae formation 1

Pain Management

• Use validated pain assessment tools at least once daily 2
• Provide adequate background simple analgesia (paracetamol, NSAIDs) to ensure comfort at rest 1, 2
• Add opiates as required, delivered enterally, by patient-controlled analgesia (PCA), or via infusion for moderate-to-severe pain 1, 2
• If using morphine-based regimens, carefully monitor level of consciousness, respiratory rate, and oxygen saturation 1

Note: Involvement of the hands by SJS/TEN may limit the ability to operate a PCA device 1

Nutrition Support

• Provide continuous enteral nutrition throughout the acute phase, either orally or via nasogastric feeding when oral intake is precluded by buccal mucositis 2, 3
• Deliver 20-25 kcal/kg daily during the catabolic phase 2, 3
• Increase to 25-30 kcal/kg daily during the anabolic recovery phase 2

Additional Supportive Measures

• Administer low molecular weight heparin as prophylactic anticoagulation for immobile patients unless contraindicated 1, 2, 3
• Provide proton pump inhibitor to protect against upper gastrointestinal stress ulceration if enteral nutrition cannot be established 1, 2, 3
• Consider recombinant human G-CSF for neutropenic patients to resist infectious complications and potentially enhance re-epithelialization 1, 2

Airway Management

• Respiratory symptoms and hypoxemia on admission should prompt urgent discussion with an intensivist and rapid transfer to ICU or burn center 2, 4
• Perform fibreoptic bronchoscopy to identify bronchial involvement, evaluate prognosis, investigate pneumonitis, and mechanically remove sloughed bronchial epithelium 2, 4

Systemic Immunomodulatory Therapy

Cyclosporine (Strongest Evidence for Mortality Reduction)

• Cyclosporine 3 mg/kg daily for 10 days, tapered over 1 month, has shown benefit in multiple studies with reduced mortality compared to predicted rates 2, 4, 3, 6
• Alternative dosing: 3-5 mg/kg/day for 10-14 days, either alone or in combination with corticosteroids 6

Systemic Corticosteroids (Controversial but Widely Used)

• If using corticosteroids, start early (preferably within 72 hours of onset) with IV methylprednisolone 0.5-1 mg/kg or oral prednisolone 1-2 mg/kg/day 2, 4, 3, 6
• Taper rapidly within 7-10 days once response is achieved 3, 6
• Evidence is mixed: Some studies show benefit when started early, while others show no mortality benefit 7, 8

TNF-Alpha Inhibitors (Emerging Evidence)

• Etanercept may reduce disease-specific mortality compared to corticosteroids (25 mg, or 50 mg if weight >65 kg, twice weekly until skin lesions healed), though confidence intervals were wide 7, 8
• Serious adverse events (sepsis, respiratory failure) occurred in 5 of 48 participants with etanercept versus 9 of 43 with corticosteroids 7

Intravenous Immunoglobulin (IVIG) - Not Recommended

• IVIG has equivocal evidence—pooled analysis showed no mortality benefit (OR 1.00,95% CI 0.58-1.75) 2, 7
• Typical dosing when used: 0.2-0.5 g/kg cumulative dose over 3 days, or continuous infusion 0.75 g/kg/day for 4 days (total dose 3 g/kg) 7

Thalidomide - Contraindicated

• Thalidomide should be avoided—it was associated with excess deaths in one randomized trial 2, 8

Nuance on Systemic Therapy: The British Journal of Dermatology guidelines (2016) and recent evidence favor cyclosporine or early corticosteroids over IVIG. The most recent Cochrane review (2022) found low-certainty evidence for etanercept potentially reducing mortality compared to corticosteroids, but this requires confirmation in larger trials 7.

Discharge Planning and Follow-up

• Provide written information about the culprit drug(s) to avoid and any potentially cross-reactive medications 2, 4, 3
• Encourage patients to wear a MedicAlert bracelet bearing the name of the culprit drug 2, 4, 3
• Document drug allergy in the patient's medical records and inform all healthcare providers involved in their care 2, 4, 3
• Report the adverse drug reaction to national pharmacovigilance authorities 2, 4, 3
• Arrange dermatology outpatient clinic appointment within a few weeks of discharge 4, 3
• Schedule ophthalmology follow-up to monitor for chronic ocular complications (50-63% develop late complications including severe dry eyes and trichiasis) 1, 3
• Inform patients about potential fatigue and lethargy for several weeks following discharge and the need for convalescence 2

Special Considerations for Pediatric Patients

• Infection causes up to 50% of pediatric SJS/TEN cases—test for infective triggers and consult infectious disease team in all pediatric cases 2
• Manage pediatric patients in age-appropriate specialist units with pediatric intensivists and skin loss specialists 2
• High-risk children need quicker transfer to specialized care 2