What is the treatment for Stevens-Johnson Syndrome?

Treatment of Stevens-Johnson Syndrome

Patients with Stevens-Johnson Syndrome (SJS) should be immediately admitted to a burn center or intensive care unit with experience in managing SJS, with prompt discontinuation of the culprit drug and implementation of supportive care as the cornerstone of management. 1, 2

Initial Assessment and Management

• Immediate actions:

  • Identify and discontinue the suspected causative drug 2
  • Calculate SCORTEN score to predict mortality risk 1
  • Transfer to burn center or ICU if >10% body surface area involvement 1
  • Consult dermatology for confirmation of diagnosis 1

• Supportive care priorities:

  • Maintain barrier nursing in a side room with controlled humidity
  • Adjust ambient temperature to 25-28°C 1
  • Place patient on a pressure-relieving mattress 1
  • Establish fluid and electrolyte balance (requirements are lower than for thermal burns) 2

Wound Management

• Skin care approach:

  • Handle skin carefully to minimize shearing forces and epidermal detachment 1
  • Apply bland emollients frequently to support barrier function 1
  • Use non-adherent dressings on denuded dermis 2
  • Consider silver-releasing antimicrobial dressings changed every 3 days 3
  • Leave detached epidermis in situ as a biological dressing 2

• Wound cleaning:

  • Gently cleanse wounds using warmed sterile water, saline, or dilute chlorhexidine 2
  • Apply greasy emollient (50% white soft paraffin with 50% liquid paraffin) over entire epidermis 2

Systemic Therapy

• Corticosteroids:

  • For immune checkpoint inhibitor-induced SJS: IV methylprednisolone 0.5-1 mg/kg, then convert to oral corticosteroids and taper over at least 4 weeks 1
  • For severe cases (≥10% BSA): IV methylprednisolone 1-2 mg/kg 1
  • Note: The traditional prohibition of corticosteroids in SJS is not relevant for immune-mediated cases 1

• Additional therapies for severe or steroid-unresponsive cases:

  • Consider IVIG or cyclosporine A 1, 4
  • Recent evidence suggests early IVIG administration may improve outcomes 3

Management of Mucosal Surfaces

• Ocular care:

  • Arrange ophthalmology consultation within 24 hours 2
  • Apply preservative-free lubricants every 2 hours 2
  • Consider topical antibiotics and corticosteroid drops under ophthalmologist supervision 2
  • Prevent corneal exposure in unconscious patients with moisture chambers 1

• Oral care:

  • Clean mouth daily with warm saline mouthwashes 2
  • Consider topical corticosteroids (betamethasone sodium phosphate 0.5 mg in 10 mL water as rinse-and-spit) 1
  • Use benzydamine hydrochloride rinse every 3 hours, particularly before eating 2

• Urogenital care:

  • Examine urogenital tract as part of initial assessment 1
  • Apply white soft paraffin to urogenital skin/mucosae every 4 hours 1
  • Use silicone dressings on eroded areas to reduce pain and prevent adhesions 1
  • Consider catheterization to prevent urethral strictures 1

Pain Management

• Follow WHO analgesic ladder principles 2
• Consider opiate-based regimens for moderate-to-severe pain 2
• Patient-controlled analgesia may be beneficial 2

Prevention and Management of Complications

• Infection prevention:

  • Avoid prophylactic systemic antibiotics 2
  • Monitor for signs of sepsis (leading cause of mortality) 4
  • Treat infections promptly when clinically indicated 2

• Nutritional support:

  • Provide supplemental tube feeding as needed 3
  • Ensure adequate caloric intake to support healing 2

Follow-up Care

• Provide written information about drugs to avoid 2
• Arrange follow-up with dermatology, ophthalmology, and other specialists 2
• Monitor for long-term sequelae including respiratory, ocular, cutaneous, and urogenital complications 2

Common Pitfalls to Avoid

1. Delayed transfer to specialized care - Early transfer to burn centers is associated with improved outcomes 3
2. Continued administration of culprit drug - Immediate discontinuation is essential 2
3. Excessive fluid administration - SJS patients require less fluid than predicted by burn formulas 2
4. Prophylactic antibiotics - May increase skin colonization with resistant organisms 1
5. Aggressive debridement - Conservative wound management is preferred initially 1

The treatment approach should be multidisciplinary, involving dermatology, intensive care, ophthalmology, and specialist skincare nursing, with additional input from other specialties as needed based on organ involvement 1.