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

Stevens-Johnson Syndrome Treatment

Immediately discontinue all suspected culprit medications and transfer the patient to a specialized burn unit or ICU within hours of diagnosis, as delayed transfer significantly increases mortality risk. 1, 2

Immediate Life-Saving Actions

• Stop all potential causative drugs immediately upon suspicion of SJS/TEN—this is the single most critical intervention that directly impacts survival 1, 3, 2
• Calculate SCORTEN within the first 24 hours to predict mortality risk (scores range 0-7, with mortality from 1% to 99%) 1, 2
• Transfer patients with >10% body surface area (BSA) epidermal detachment to a specialized burn center or ICU with multidisciplinary expertise 1, 2
• Document all medications taken in the previous 2 months, including over-the-counter and herbal products, with exact start dates 1, 3

Specialized Care Environment

• Barrier-nurse patients in a temperature-controlled side room (25-28°C) on a pressure-relieving mattress with humidity control 1, 2
• Coordinate care through a multidisciplinary team led by a dermatologist or burn specialist, including intensive care physicians, ophthalmologists, and specialist skincare nurses 1, 3, 2

Supportive Care Framework

Fluid Management

• Establish adequate intravenous fluid replacement guided by urine output and hemodynamic parameters 1, 2
• Avoid overaggressive fluid resuscitation which causes pulmonary, cutaneous, and intestinal edema 1, 2
• Monitor fluid balance with urinary catheterization when clinically indicated 1, 2

Wound Care Protocol

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

Infection Prevention and Management

• Do NOT use prophylactic systemic antibiotics—indiscriminate administration increases skin colonization with resistant organisms, particularly Candida albicans 1, 3, 2
• Take swabs for bacterial and candidal culture from three lesional areas on alternate days 1, 2
• Institute targeted antimicrobial therapy only when clinical signs of infection appear (confusion, hypotension, reduced urine output, reduced oxygen saturation) 1, 2
• Monitor for rising C-reactive protein and neutrophilia as indicators of sepsis 1

Nutrition Support

• Provide continuous enteral nutrition throughout the acute phase, delivering 20-25 kcal/kg daily during the catabolic phase 1, 3
• Increase to 25-30 kcal/kg daily during the anabolic recovery phase 1
• Consider nasogastric feeding when oral intake is precluded by buccal mucositis 1

Pain Management

• Use validated pain assessment tools at least once daily 1, 2
• Provide adequate background analgesia with simple analgesics plus additional opioid analgesia for breakthrough pain 4, 1
• Consider patient-controlled analgesia or sedation/general anesthesia for dressing changes 2

Mucosal Management

Ocular Care (Critical Priority)

• Arrange ophthalmology consultation within 24 hours of diagnosis with daily examinations throughout the acute phase 1, 3, 2
• Apply preservative-free lubricant eye drops every 2 hours throughout the acute illness 1
• Perform daily ocular hygiene by an ophthalmologist or ophthalmically trained nurse to remove inflammatory debris and break down conjunctival adhesions 1
• Use topical antibiotics when corneal fluorescein staining or ulceration is present 1
• Consider topical corticosteroid drops under ophthalmologist supervision to reduce ocular surface damage 1
• Consider amniotic membrane transplantation in the acute phase for significantly better visual outcomes 1

Oral Care

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

Urogenital Care

• Perform regular examination of urogenital tract during acute illness 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

Systemic Immunomodulatory Therapy

The evidence for systemic immunomodulation remains controversial, but early intervention within 72 hours appears beneficial:

• Cyclosporine 3 mg/kg daily for 10 days, tapered over 1 month has shown benefit with reduced mortality compared to predicted rates 1, 3
• IV methylprednisolone 0.5-1 mg/kg (or equivalent) if started within 72 hours of onset, converting to oral corticosteroids on response with tapering over at least 4 weeks 4, 1, 3
  • For immune checkpoint inhibitor-induced SJS/TEN (grade 3), use 0.5-1 mg/kg 4
  • For grade 4 disease, use 1-2 mg/kg 4
• IVIG or cyclosporine may be considered in severe or steroid-unresponsive cases 4
• Note: IVIg evidence is equivocal—pooled analysis showed no mortality benefit (OR 1.00,95% CI 0.58-1.75) 1
• Avoid thalidomide—it was associated with excess deaths in one randomized trial 1

Additional Supportive Measures

• Administer low molecular weight heparin as prophylactic anticoagulation for immobile patients 1, 3
• Provide proton pump inhibitor if enteral nutrition cannot be established 1, 3
• Consider recombinant human G-CSF for neutropenic patients 1

Airway Management

• Respiratory symptoms and hypoxemia on admission require urgent discussion with an intensivist and rapid transfer to ICU 1
• Perform fibreoptic bronchoscopy to identify bronchial involvement, evaluate prognosis, and mechanically remove sloughed bronchial epithelium 1

Discharge Planning and Follow-up

• Provide written information about the culprit drug(s) to avoid permanently and any potentially cross-reactive medications 1, 3
• Encourage patients to wear a MedicAlert bracelet bearing the name of the culprit drug 1, 3
• Document drug allergy in the patient's medical records and inform all healthcare providers 1, 3
• Report the adverse drug reaction to pharmacovigilance authorities 1, 3
• Arrange dermatology follow-up within weeks of discharge 3
• Schedule ophthalmology follow-up to monitor for chronic ocular complications 3
• Inform patients about potential fatigue and lethargy for several weeks following discharge 1

Critical Pitfalls to Avoid

• Delayed transfer to a specialized unit significantly increases mortality risk—transfer within hours, not days 1, 3, 2
• Failure to involve ophthalmology within 24 hours leads to permanent visual impairment 1, 3
• Continued use of the culprit medication will worsen the condition and increase mortality 1
• Overaggressive fluid resuscitation causes complications 1, 2
• Indiscriminate prophylactic antibiotics increase resistant organism colonization 1, 3, 2
• Failure to recognize and treat sepsis, which is the most common cause of death in SJS/TEN 2

Special Considerations for Immune Checkpoint Inhibitor-Induced SJS/TEN

• Permanently discontinue immune checkpoint inhibitor therapy 4
• The usual prohibition of corticosteroids for SJS does not apply here, as the underlying mechanism is T-cell immune-directed toxicity requiring adequate immune suppression 4
• Admit immediately to a burn unit or ICU with consulted dermatology and wound care services 4
• Consider pain or palliative consultation in patients presenting with DRESS manifestations 4

Pediatric Considerations

• Infection causes up to 50% of pediatric SJS/TEN cases—test for infective triggers (Mycoplasma pneumoniae, HSV) and consult infectious disease team in all pediatric cases 1
• Manage in age-appropriate specialist units with pediatric intensivists 1
• Consider targeted antibiotics as appropriate (e.g., azithromycin for mycoplasma) 1