What is the treatment for Steven Johnson syndrome?

Treatment of Stevens-Johnson Syndrome

The treatment of Stevens-Johnson Syndrome (SJS) requires immediate transfer to a specialized care setting such as a burn center or intensive care unit, with prompt withdrawal of any suspected causative medications and implementation of supportive care measures including wound care, fluid management, and pain control. 1, 2

Initial Management and Setting

• Transfer to specialized care: Patients with SJS/TEN should be managed in specialized units:

  • Adults with >10% body surface area (BSA) involvement should be admitted to a burn center or ICU 1, 2
  • Children should be managed in age-appropriate specialist units with multidisciplinary team support 1
  • Delay in transfer to specialized care adversely affects outcomes 1

• Immediate interventions:

  • Withdraw all suspected causative drugs immediately 1, 2
  • Document all medications taken in previous 2 months 2
  • Calculate SCORTEN within first 24 hours to predict mortality risk 2

Wound Care and Skin Management

• Gentle wound cleansing: Use warmed sterile water, saline, or dilute chlorhexidine (1/5000) 1

• Topical treatment:

  • Apply greasy emollient (50% white soft paraffin with 50% liquid paraffin) over entire epidermis 1
  • Consider aerosolized formulations to minimize shearing forces 1
  • Apply topical antimicrobials only to sloughy areas (guided by local microbiological advice) 1
  • Consider silver-containing products/dressings (limited use if extensive areas affected) 1

• Blister management:

  • Leave detached epidermis in situ as a biological dressing 1
  • Decompress blisters by piercing and expression or aspiration 1
  • Apply non-adherent dressings (e.g., Mepitel™ or Telfa™) to denuded dermis 1
  • Use secondary foam or burn dressings to collect exudate 1

Systemic Therapy Options

• Corticosteroids:

  • Consider IV methylprednisolone 1-2 mg/kg for severe cases (≥10% BSA involvement) 2
  • Use during progression phase and withdraw as soon as possible to reduce infection risk 3

• Cyclosporine:

  • Emerging evidence supports cyclosporine at 3 mg/kg daily for 7-14 days 1, 4
  • Multiple studies show reduced mortality compared to predicted rates 1

• Intravenous Immunoglobulin (IVIG):

  • May be considered for severe cases 2, 5
  • Most effective when combined with corticosteroids for SJS/TEN overlap and TEN 4

Supportive Care

• Pain management:

  • Provide adequate background analgesia following WHO analgesic ladder principles 1, 2
  • Consider opiate-based regimens for moderate-to-severe pain 2

• Fluid management:

  • Ensure adequate IV fluid replacement through non-lesional skin 2
  • Monitor fluid balance carefully (requirements are lower than predicted by burn formulas) 2

• Nutritional support:

  • Provide continuous enteral nutrition throughout acute phase 1
  • Deliver 20-25 kcal/kg daily during early catabolic phase 1
  • Increase to 25-30 kcal/kg daily during recovery phase 1
  • Use nasogastric feeding if oral intake is precluded by buccal mucositis 1

Mucosal Care

• Ocular care:

  • Arrange ophthalmology examination within 24 hours 2
  • Perform daily ophthalmological review during acute illness 2

• Oral care:

  • Consider topical corticosteroids (betamethasone sodium phosphate rinse-and-spit) 2
  • Clean mouth daily with warm saline mouthwashes 2

• Urogenital care:

  • Apply white soft paraffin to urogenital skin/mucosae every 4 hours 2
  • Consider catheterization to prevent urethral strictures 2

Infection Prevention and Management

• Monitoring:

  • Monitor for sepsis (may be masked by disease-associated fever) 2
  • Obtain bacterial swabs from lesional skin for culture 2

• Antibiotic use:

  • Avoid prophylactic antibiotics without clinical signs of infection 2
  • Consider antibacterials for prophylaxis if corticosteroids are used 3

Follow-up and Prevention

• Discharge planning:

  • Provide written information about drugs to avoid 1, 2
  • Encourage wearing a MedicAlert bracelet 1, 2
  • Report episode to pharmacovigilance authorities 1, 2

• Long-term follow-up:

  • Arrange follow-up with dermatology, ophthalmology, and other specialists 2
  • Monitor for potential complications including respiratory, ocular, cutaneous, and urogenital sequelae 1, 2
  • Refer to specialist drug allergy service, especially where avoidance of causal drug is medically compromising 1

Special Considerations

• High-risk patients requiring more intensive monitoring:

  • Extensive epidermal loss (>70% BSA)
  • High initial SCORTEN
  • Medication-induced SJS/TEN
  • Underlying malignancy or previous stem cell transplantation 1

• Recovery timeline:

  • Healing typically takes 3-6 weeks depending on extent and severity 3
  • Skin lesions usually heal without scars, but mucosal scarring is a frequent complication 3