What is the treatment for Stevens-Johnson Syndrome?

Treatment of Stevens-Johnson Syndrome

The treatment of Stevens-Johnson Syndrome (SJS) requires immediate withdrawal of the suspected causative drug, transfer to a specialized unit (burn center or ICU for adults with >10% body surface area involvement), and implementation of comprehensive supportive care with a multidisciplinary approach. 1

Initial Management

• Immediate actions:

  • Identify and withdraw all suspected causative medications 2
  • Calculate SCORTEN within first 24 hours to predict mortality risk 1
  • Document extent of skin involvement using body mapping 1
  • Transfer to appropriate specialized unit (burn center or ICU) 1

• Vital supportive care:

  • Maintain ambient temperature between 25-28°C 1
  • Place patient on pressure-relieving mattress 1
  • Handle skin carefully to minimize shearing forces 1

Wound Management

• Skin care protocol:
  • Cleanse wounds regularly using warmed sterile water, saline, or dilute chlorhexidine (1/5000) 1
  • Leave detached epidermis in situ as a biological dressing 1
  • Decompress blisters by piercing and expression or aspiration of fluid 1
  • Apply greasy emollient (50% white soft paraffin with 50% liquid paraffin) over entire epidermis 1
  • Apply non-adherent dressings to denuded dermis (e.g., Mepitel or Telfa) 1

Fluid and Nutritional Support

• Fluid management:

  • Use crystalloid solutions for fluid resuscitation (balanced crystalloids preferred over normal saline) 1
  • Avoid overaggressive fluid resuscitation to prevent pulmonary, cutaneous, and intestinal edema 1
  • Fluid requirements are lower than predicted by burn formulas 1

• Nutritional support:

  • Provide continuous enteral nutrition throughout the acute phase 3
  • Deliver 20-25 kcal/kg daily during early catabolic phase 3
  • Increase to 25-30 kcal/kg daily during recovery phase 3
  • Use nasogastric feeding if oral route is precluded by buccal mucositis 3

Pain Management

• Analgesia protocol:
  • Ensure adequate background simple analgesia for comfort at rest 3
  • Add opiates as required (delivered enterally, via patient-controlled analgesia, or infusion) 3
  • Use morphine-based regimen for moderate-to-severe pain uncontrolled by simple analgesia 3
  • Monitor level of consciousness, respiratory rate, and oxygen saturation when using opiates 3

Pharmacological Interventions

• Immunomodulatory treatments to consider:
  • Cyclosporine at 3 mg/kg daily for 7-14 days (emerging evidence supports its use) 1, 4
  • Systemic corticosteroids (controversial but commonly used) 4, 5
  • IV immunoglobulins (may be effective by blocking Fas ligand-mediated apoptosis) 4, 5

Additional Supportive Medications

• Prophylactic measures:
  • Low molecular weight heparin for immobile patients 3
  • Proton pump inhibitor if enteral nutrition cannot be established 3
  • Consider recombinant human G-CSF for neutropenic patients 3
  • Avoid prophylactic antibiotics without clinical signs of infection 1

Ocular Management

• Eye care:
  • Early ophthalmology consultation for all patients 1
  • Regular assessment and management of ocular complications 3
  • Note that 74% of patients suffer eye involvement during acute phase 3

Monitoring and Complications

• Infection surveillance:

  • Watch for signs of systemic infection (confusion, hypotension, reduced urine output, reduced oxygen saturation) 1
  • Monitor for sepsis, which may be masked by disease-associated fever 1
  • Obtain bacterial swabs from lesional skin for culture 1

• Common complications to monitor:

  • Ocular complications (may develop in 63% of patients with acute eye involvement) 3
  • Cutaneous complications 1
  • Renal complications 2
  • Respiratory and hepatic involvement 2

Long-term Follow-up

• Provide written information about drugs to avoid 1
• Encourage wearing a MedicAlert bracelet 1
• Report episode to pharmacovigilance authorities 1
• Refer to specialist drug allergy service 1
• Arrange follow-up with dermatology, ophthalmology, and other specialists 1

Pitfalls and Caveats

• Delayed recognition and continued exposure to causative drug increases mortality
• Overaggressive fluid resuscitation can lead to complications
• Systemic corticosteroids may enhance risk of infection while suppressing progression 5
• Prophylactic antibiotics without clinical signs of infection may increase skin colonization, particularly with Candida albicans 1
• Neither the severity of systemic disease nor grade of acute ocular disease predicts late ocular complications 3