What is the recommended treatment and management for Stevens‑Johnson syndrome?

Treatment of Stevens-Johnson Syndrome

Immediately discontinue all suspected culprit drugs and transfer patients with >10% body surface area (BSA) epidermal detachment to a specialized burn center or ICU within hours of diagnosis, as delayed transfer significantly increases mortality risk. 1

Immediate Assessment and Triage

• Calculate SCORTEN within the first 24 hours to predict mortality risk (scores 0-7 correlate with mortality from 1% to 99%) 1, 2
• Document exact date of rash onset, all medications taken in preceding 2 months including over-the-counter products, and record vital signs with oxygen saturation 2
• Obtain skin biopsy showing confluent epidermal necrosis with subepidermal vesicle formation to confirm diagnosis 2
• Arrange ophthalmology consultation within 24 hours of diagnosis—this is mandatory and delays lead to permanent visual impairment 1, 2

Specialized Care Environment

Patients must be barrier-nursed in a temperature-controlled room (25-28°C) on a pressure-relieving mattress to prevent hypothermia and reduce infection risk. 1, 2

• Transfer without delay to centers with multidisciplinary teams including dermatology, intensive care, burn surgery, and ophthalmology 2
• Care should be coordinated by a specialist in skin failure with core team members including intensive care physicians, ophthalmologists, and specialist skincare nurses 2, 3

Skin and Wound Management

Handling and Cleansing

• Handle skin with extreme gentleness to minimize shearing forces that cause further epidermal detachment 1, 2, 3
• Regularly cleanse wounds by gently irrigating with warmed sterile water, saline, or chlorhexidine (1/5000) 1, 2
• Day-to-day care should be undertaken by specialist nurses familiar with skin fragility disorders 1

Topical Treatment Protocol

• Apply greasy emollient (50% white soft paraffin with 50% liquid paraffin) over the entire epidermis every 2-4 hours, including denuded areas 1, 2, 3
• Leave detached epidermis in situ to act as a biological dressing 1, 2, 3
• Decompress blisters by piercing and expression or aspiration of fluid 2
• Apply nonadherent dressings to denuded dermis with secondary foam or burn dressings to collect exudate 2
• Consider high-strength topical corticosteroids on affected skin areas 2

Surgical Approach

• Consider surgical debridement of detached epidermis followed by biosynthetic xenograft or allograft only after failure of conservative management, characterized by clinical deterioration, extension of epidermal detachment, local sepsis, delayed healing, or wound conversion 1

Systemic Immunomodulatory Therapy

Administer IV methylprednisolone 0.5-1 mg/kg (FDA-approved for severe erythema multiforme/Stevens-Johnson syndrome) if started within 72 hours of onset, converting to oral corticosteroids on response with tapering over at least 4 weeks. 2, 3, 4, 5

• Alternative option: Cyclosporine 3-5 mg/kg daily for 10-14 days, tapered over 1 month, which has shown reduced mortality compared to predicted rates 2, 3, 5
• For immune-checkpoint-inhibitor-induced SJS: use methylprednisolone 0.5-1 mg/kg for Grade 3 disease or 1-2 mg/kg for Grade 4 disease, with permanent discontinuation of the offending agent 2
• IVIG may be considered in severe disease or inadequate response to corticosteroids, though pooled analysis showed no mortality benefit (OR 1.00,95% CI 0.58-1.75) 2
• Avoid thalidomide—it was associated with excess deaths in one randomized trial 2

Infection Prevention and Management

Do NOT administer prophylactic systemic antibiotics, as 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 2
• Monitor for clinical signs of infection: confusion, hypotension, reduced urine output, reduced oxygen saturation, increased skin pain, rising C-reactive protein, and neutrophilia 1, 2
• Institute targeted antimicrobial therapy ONLY when clinical signs of infection are present 1, 2, 3
• Watch for monoculture of organisms on culture swabs from multiple sites, indicating increased likelihood of invasive infection 3

Mucosal Management

Ophthalmologic Care (Critical Priority)

• 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 2, 3
• Use topical antibiotics when corneal fluorescein staining or ulceration is present 1, 2
• Consider topical corticosteroid drops under ophthalmologist supervision to reduce ocular surface damage 1, 2, 3
• Consider amniotic membrane transplantation in the acute phase for significantly better visual outcomes 2

Oral Care

• Apply white soft paraffin ointment to the lips immediately, then every 2 hours throughout the acute illness 2, 3
• Use anti-inflammatory oral rinse containing benzydamine hydrochloride every 3 hours, particularly before eating 1, 2
• Use antiseptic oral rinse twice daily to reduce bacterial colonization 1, 2
• 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

• Use urinary catheterization when urogenital involvement causes dysuria or retention, or to monitor output 1
• Apply white soft paraffin ointment to urogenital skin and mucosae every 4 hours 1
• Consider vaginal dilators or tampons wrapped in Mepitel to prevent vaginal synechiae formation 1

Supportive Care Framework

Fluid and Nutrition Management

• Provide careful fluid resuscitation to prevent end-organ hypoperfusion while avoiding overaggressive replacement that leads to pulmonary, cutaneous, and intestinal edema 2, 3
• Monitor fluid balance with regular assessment of vital signs, urine output, and electrolytes 1, 2
• Deliver continuous enteral nutrition: 20-25 kcal/kg daily during the catabolic phase, increasing to 25-30 kcal/kg during recovery 1, 2
• Consider nasogastric feeding when oral intake is precluded by buccal mucositis 2

Pain Management

• Use validated pain assessment tools at least once daily 2
• Provide adequate background analgesia with simple (non-opioid) analgesics to ensure comfort at rest 2
• Add opioid analgesia for breakthrough pain episodes 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

Airway and Respiratory Management

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

Special Considerations for Pediatric Patients

• Infection causes up to 50% of pediatric SJS/TEN cases; test for infective triggers (Mycoplasma pneumoniae, Herpes simplex virus) and consult infectious disease team in all pediatric cases 1, 2, 3
• Manage pediatric patients in age-appropriate specialist units with pediatric intensivists and skin loss specialists 2, 3
• Mortality is lower in children than adults (0-8.5% depending on severity) but recurrence is more common (up to 18% of cases) 1
• Consider targeted antibiotics as appropriate (e.g., azithromycin for mycoplasma) 2

Discharge Planning and Long-Term Follow-Up

• Provide written information about the culprit drug(s) to avoid and any potentially cross-reactive medications 1, 2, 3
• Encourage patients to wear a MedicAlert bracelet bearing the name of the culprit drug 1, 2, 3
• Document drug allergy in the patient's medical records and inform all healthcare providers involved in their care 1, 2, 3
• Report the adverse drug reaction to national pharmacovigilance authorities 1, 2, 3
• Inform patients about potential fatigue and lethargy for several weeks following discharge and the need for convalescence 1
• Consider referral to support groups such as SJS Awareness U.K. 1

Critical Pitfalls to Avoid

• Delayed recognition and transfer to specialized care significantly increases mortality risk—transfer should occur within hours, not days 2, 3
• Failure to involve ophthalmology within 24 hours leads to permanent visual impairment—this is the most common long-term sequela 1, 2, 3
• Indiscriminate use of prophylactic antibiotics increases resistant organism colonization, particularly Candida 1, 2, 3
• Overaggressive fluid resuscitation causes pulmonary, cutaneous, and intestinal edema 2, 3
• Continued use of the culprit medication will worsen the condition and increase mortality 2, 3
• Examining the patient without awareness of skin fragility can cause further epidermal detachment—warn all attending clinicians unfamiliar with the condition 1