What is the appropriate management and treatment for Stevens-Johnson syndrome?

Management of Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis

Immediately discontinue the suspected culprit drug, transfer patients with >10% body surface area (BSA) epidermal loss to a burn center or intensive care unit with SJS/TEN experience, and initiate comprehensive multidisciplinary supportive care as the cornerstone of treatment. 1

Initial Assessment and Triage

Severity Assessment

• Calculate SCORTEN on admission to predict mortality risk (parameters include age >40 years, malignancy, tachycardia >120 bpm, initial BSA detachment >10%, serum urea >10 mmol/L, serum glucose >14 mmol/L, serum bicarbonate <20 mmol/L) 1
• Assess BSA involvement to determine appropriate care setting 1
• Document all suspected medications and stop them immediately 1

Transfer Criteria

• Transfer to burn center or specialized ICU if: BSA epidermal loss >10%, respiratory symptoms with hypoxemia, or clinical deterioration 1
• Rapid admission to specialized centers improves survival; delayed transfer increases mortality 1
• Coordinate care through a multidisciplinary team including dermatology/plastic surgery, intensive care, ophthalmology, and specialist nursing 1

Supportive Care Measures

Environmental Control

• Barrier-nurse in side room with controlled humidity 1
• Maintain ambient temperature between 25-28°C 1
• Use pressure-relieving mattress 1
• Practice antishear handling techniques to minimize epidermal detachment 1

Fluid Management

• Avoid overaggressive resuscitation (causes pulmonary, cutaneous, and intestinal edema) 1
• Use modified formula: body weight/% BSA epidermal detachment for fluid requirements (lower than Parkland formula used for burns) 1

Nutritional Support

• Initiate early enteral nutrition (oral or nasogastric if buccal mucositis prevents oral intake) 1
• Provide 20-25 kcal/kg daily during catabolic phase 1
• Increase to 25-30 kcal/kg daily during anabolic recovery phase 1
• Use silicone nasogastric tube if needed 1

Pain Management

• Provide adequate analgesia for significant cutaneous pain 1

Skin and Wound Management

General Skin Care

• Cleanse wounds and intact skin with warmed sterile water, saline, or chlorhexidine (1:5000) 1
• Apply greasy emollient (50% white soft paraffin with 50% liquid paraffin) over entire epidermis including denuded areas 1
• Consider aerosolized formulations to minimize shearing forces 1
• Avoid preparations containing sensitizers or irritants 1

Wound Dressing

• Leave detached lesional epidermis in situ as biological dressing 1
• Decompress blisters by piercing and expressing fluid 1
• Apply nonadherent dressings (Mepitel™ or Telfa™) to denuded dermis 1
• Use secondary foam or burn dressing (Exu-Dry™) to collect exudate 1
• Apply topical antimicrobial agents to sloughy areas only (choice guided by local microbiology) 1
• Consider silver-containing products/dressings with caution due to absorption risk 1

Surgical Approach (for TEN >30% BSA with deterioration)

• Remove necrotic/loose infected epidermis under general anesthetic 1
• Clean wounds with topical antimicrobial (betadine or chlorhexidine) 1
• Consider debridement with Versajet™ 1
• Physiological closure with Biobrane/allograft/xenograft for early presentation with noninfected confluent areas 1

Ocular Management

Daily Ophthalmology Assessment

• Perform daily eye examination during acute illness 1
• Maintain ocular surface lubrication and conjunctival hygiene throughout acute phase 1
• Ensure prevention of corneal exposure in semiconscious/unconscious patients 1

Specific Interventions

• Administer broad-spectrum topical antibiotic prophylaxis (moxifloxacin drops four times daily) if corneal fluorescein staining or frank ulceration present 1
• Consider topical corticosteroids to ameliorate conjunctival inflammation (though evidence is limited) 1
• Arrange ophthalmology follow-up within weeks of discharge, as late complications may develop regardless of acute severity 1

Oral Care

Daily Oral Protocol

• Perform daily oral review during acute illness 1
• Apply white soft paraffin ointment to lips every 2 hours 1
• Clean mouth daily with warm saline mouthwashes or oral sponge 1
• Use benzydamine hydrochloride anti-inflammatory oral rinse/spray every 3 hours, particularly before eating 1
• Apply chlorhexidine antiseptic oral rinse twice daily 1
• Use potent topical corticosteroid mouthwash (betamethasone sodium phosphate) four times daily 1

Urogenital Care

Daily Urogenital Protocol

• Perform daily urogenital review during acute illness 1
• Apply white soft paraffin ointment to urogenital skin and mucosae every 4 hours 1
• Use potent topical corticosteroid ointment once daily to involved but noneroded surfaces 1
• Apply silicone dressing (Mepitel) to eroded areas 1

Airway Management

• If respiratory symptoms or hypoxemia on admission: Immediately discuss with intensivist and rapidly transfer to ICU or burn center for fiberoptic bronchoscopy 1

Immunomodulatory Therapy

Current Evidence Status

• No international consensus exists on optimal immunomodulatory treatment 2, 3, 4
• Systemic corticosteroids remain most common first-line treatment despite uncertain efficacy 4, 5
• Increasing evidence supports cyclosporine and TNF-α inhibitors for reducing mortality 4, 5
• If active therapy instituted: Administer under supervision of specialist skin failure multidisciplinary team, ideally within clinical research/case registry context 1

Emerging Options

• Cyclosporine: Studies show 3 mg/kg daily for 7-10 days with tapering may reduce mortality 1
• TNF-α inhibitors: Gaining attention for potential efficacy 4, 5
• Intravenous immunoglobulin, plasmapheresis, and JAK inhibitors: Used in select cases 5

Discharge Planning and Follow-up

Patient Education

• Provide written information about drug(s) to avoid, including related medications that may cross-react 1
• Encourage MedicAlert bracelet/amulet with culprit drug name 1
• Document drug allergy in patient notes and inform all involved physicians, especially GP 1
• Warn about avoiding over-the-counter medications with unclear constituents 1

Reporting and Follow-up

• Report episode to national pharmacovigilance authorities (Yellow Card Scheme in UK) 1
• Arrange dermatology outpatient appointment within weeks of discharge 1
• Arrange ophthalmology follow-up if ocular involvement occurred 1
• Refer to unit with appropriate subspecialty interest 1
• Counsel patient/family about expected fatigue and lethargy for several weeks post-discharge 1
• Monitor for psychological problems including depression 1

Long-term Monitoring

• Do not perform routine drug hypersensitivity testing following SJS/TEN episode 1
• Seek specialist advice on hypersensitivity testing only if: culprit drug unknown, medication avoidance detrimental to individual, or accidental exposure possible 1
• Monitor for chronic complications (ocular, cutaneous, mucosal, psychological) that may develop weeks to months after acute episode 1
• Recurrence is more common in children (up to 18%) than adults, particularly when infection-triggered 1

Critical Pitfalls to Avoid

• Overaggressive fluid resuscitation leads to complications; use modified formula, not standard burn formulas 1
• Delayed transfer to specialized centers increases mortality 1
• Failure to arrange ophthalmology follow-up: Late ocular complications can develop regardless of acute severity and may not appear until months later 1
• Inadequate documentation of drug allergy across all care settings 1
• Shearing forces during patient handling and topical applications worsen epidermal detachment 1