What is the appropriate management and treatment for a patient suspected of having Stevens-Johnson syndrome?

Management of Stevens-Johnson Syndrome

Immediately discontinue all suspected culprit medications and transfer the patient to a specialized burn unit or ICU within hours—this is the single most critical intervention that directly impacts survival. 1, 2, 3

Immediate Recognition and Triage

• Calculate SCORTEN within the first 24 hours to predict mortality risk (scores 0-7 correlate with mortality from 1% to 99%) and guide intensity of care 2, 3, 4
• Transfer any patient with >10% body surface area epidermal detachment to a specialized burn center or ICU without delay—delayed transfer significantly increases mortality 1, 2, 3
• Look for the diagnostic triad: fever, purpuric macules or flat atypical targets with epidermal detachment, and painful erosive mucositis affecting at least two mucosal sites (oral, ocular, genital) 2, 5, 6
• Document positive Nikolsky sign (lateral pressure causes skin slippage) and record exact date of rash onset 2, 4

Specialized Care Environment

• Barrier-nurse the patient in a temperature-controlled room at 25-28°C on a pressure-relieving mattress to prevent hypothermia and reduce infection risk 2, 3
• Assemble a multidisciplinary team including dermatology, intensive care physicians, burn surgeons, ophthalmologists, and specialist skincare nurses within the first 24 hours 2, 3, 5

Supportive Care Framework

Fluid and Hemodynamic Management

• Establish intravenous fluid replacement guided by urine output (target 0.5-1 mL/kg/hour), avoiding overaggressive resuscitation that causes pulmonary, cutaneous, and intestinal edema 1, 2, 3
• Insert urinary catheter for accurate monitoring and to prevent urethral strictures 1
• Monitor vital signs, electrolytes, and lactate levels every 4-6 hours during the acute phase 2

Wound Care Protocol

• Handle skin with extreme care using minimal contact to prevent shearing forces that cause further epidermal detachment 1, 2, 3
• Leave detached epidermis in situ to act as a biological dressing—do not debride intact blisters 2, 3
• Apply greasy emollient (50% white soft paraffin with 50% liquid paraffin) over the entire epidermis including denuded areas every 2-4 hours 1, 2, 3
• Use nonadherent dressings (such as Mepitel) on denuded dermis with secondary foam dressings to collect exudate 1, 2
• Irrigate wounds gently with warmed sterile water, saline, or dilute chlorhexidine (1:5000) once daily 2

Infection Prevention and Management

Do not use prophylactic systemic antibiotics—indiscriminate administration increases skin colonization with resistant organisms, particularly Candida albicans. 1, 2, 3

• Take swabs for bacterial and candidal culture from three lesional areas on alternate days throughout the acute phase 2, 3
• Administer targeted antimicrobial therapy only when clinical signs of infection appear: fever with hemodynamic instability, rising C-reactive protein, neutrophilia, or positive cultures 1, 2, 3
• Monitor for sepsis as the leading cause of mortality in the acute phase 5, 7

Nutrition Support

• Provide continuous enteral nutrition throughout the acute phase, delivering 20-25 kcal/kg daily during the catabolic phase 1, 2
• Increase to 25-30 kcal/kg daily during the anabolic recovery phase 2
• Use nasogastric feeding when oral intake is precluded by severe buccal mucositis 2

Pain Management

• Provide adequate background analgesia with regular paracetamol and NSAIDs (if not contraindicated) to ensure comfort at rest 2
• Add opioid analgesia (morphine or fentanyl) for breakthrough pain, particularly before wound care 2
• Use validated pain assessment tools at least once daily 2

Mucosal Management

Ocular Care (Critical Priority)

Arrange ophthalmological examination within 24 hours of diagnosis with daily reviews throughout the acute illness—50-63% of patients develop late complications including severe dry eyes, trichiasis, and vision loss. 1, 2, 3

• Apply preservative-free lubricant eye drops every 2 hours throughout the acute illness 1, 2, 3
• Perform daily ocular hygiene by an ophthalmologist or ophthalmically trained nurse to remove inflammatory debris and break down conjunctival adhesions 1, 2
• Use topical antibiotics (fluoroquinolone drops) when corneal fluorescein staining or ulceration is present 1, 2
• Consider topical corticosteroid drops (under ophthalmologist supervision) to reduce ocular surface inflammation, though evidence shows no difference in long-term outcomes 1
• Consider amniotic membrane transplantation in the acute phase for severe ocular involvement (grade 2-3), which demonstrates significantly better visual outcomes compared to medical management alone 2

Oral Care

• Apply white soft paraffin ointment to the lips immediately, then every 2 hours throughout the acute illness to reduce risk of fibrotic scars 1, 2, 3
• Use anti-inflammatory oral rinse containing benzydamine hydrochloride 0.15% every 3 hours, particularly before eating 1, 2
• Apply antiseptic oral rinse (chlorhexidine 0.2%) twice daily to reduce bacterial colonization 1, 2
• Use topical anesthetics such as viscous lidocaine 2% before meals for severe oral discomfort 1
• Treat candidal infection with nystatin oral suspension 100,000 units four times daily for 1 week 1, 2
• Consider topical corticosteroids (betamethasone sodium phosphate 0.5 mg in 10 mL water as rinse-and-spit, or clobetasol propionate 0.05% mixed with Orabase applied directly to affected areas) 1

Urogenital Care

• Examine the urogenital tract daily during the acute illness 1, 2
• Apply white soft paraffin ointment to urogenital skin and mucosae every 4 hours 1, 3
• In women, arrange early assessment by a vulval specialist for consideration of vaginal dilators or tampons wrapped in Mepitel to prevent vaginal synechiae formation 1, 2
• Catheterize all patients to prevent urethral strictures and monitor urine output 1
• Apply potent topical corticosteroid ointment once daily to involved, noneroded urogenital surfaces 1

Systemic Immunomodulatory Therapy

Cyclosporine 3 mg/kg daily for 10 days (tapered over 1 month) has shown the most consistent benefit with reduced mortality compared to predicted rates in multiple studies and is the preferred systemic therapy. 2, 3, 7

Alternative Systemic Options

• If cyclosporine is unavailable or contraindicated, use IV methylprednisolone 0.5-1 mg/kg (or oral prednisolone 1-2 mg/kg/day) started within 72 hours of onset, tapering over at least 4 weeks 1, 2, 3
• For immune checkpoint inhibitor-induced SJS/TEN specifically, use IV methylprednisolone 1-2 mg/kg as first-line therapy 1
• IVIg has equivocal evidence—pooled analysis showed no mortality benefit (OR 1.00,95% CI 0.58-1.75) and is not recommended as first-line therapy 2
• Avoid thalidomide—it was associated with excess deaths in a randomized trial 2
• TNF-α inhibitors (etanercept, infliximab) show emerging evidence of benefit but require further validation 7

Airway and Respiratory Management

• Respiratory symptoms (dyspnea, increased respiratory rate) and hypoxemia on admission require urgent intensivist discussion and rapid ICU/burn center transfer 1, 2, 3
• Perform fibreoptic bronchoscopy to identify bronchial involvement, evaluate prognosis, and mechanically remove sloughed bronchial epithelium 2
• Intubate and ventilate only in extreme circumstances due to high risk of nosocomial pneumonia and fluid overload complications 2

Additional Supportive Measures

• Administer low molecular weight heparin as prophylactic anticoagulation for immobile patients 2
• Provide proton pump inhibitor if enteral nutrition cannot be established 2
• Consider recombinant human G-CSF for neutropenic patients 2

Discharge Planning and Follow-up

• Provide written information about the culprit drug(s) to avoid and any potentially cross-reactive medications 2, 3
• Encourage patients to wear a MedicAlert bracelet bearing the name of the culprit drug 2, 3
• Document drug allergy in the patient's medical records and inform all healthcare providers involved in their care 2, 3
• Report the adverse drug reaction to national pharmacovigilance authorities (FDA MedWatch in the US) 2, 3
• Schedule ophthalmology follow-up within 1 month and then every 3-6 months during the first year to monitor for chronic ocular complications 1, 3
• Inform patients about potential fatigue and lethargy for several weeks following discharge and the need for convalescence 2
• Consider referral to support groups such as SJS Awareness UK 2

Common Pitfalls to Avoid

• Delayed recognition and transfer to specialized care significantly increases mortality—transfer within hours, not days 2, 3
• Continuing any suspected medication will worsen the condition and increase mortality—discontinue all non-essential medications immediately 2, 3
• Prophylactic antibiotics increase resistant organism colonization, particularly Candida—use targeted therapy only when infection is clinically evident 1, 2, 3
• Overaggressive fluid resuscitation causes pulmonary, cutaneous, and intestinal edema—titrate to urine output, not formulaic burn resuscitation volumes 1, 2, 3
• Failure to involve ophthalmology within 24 hours leads to permanent vision loss—neither severity of systemic disease nor grade of acute ocular disease predicts late complications, so all patients need specialist follow-up 1, 2, 3
• Aggressive debridement of detached epidermis removes the natural biological dressing—leave intact blisters and detached skin in place 2, 3