What is the management approach for a patient diagnosed with Stevens-Johnson (Toxic Epidermal Necrolysis) syndrome?

Management of Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis

The management of Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis (SJS/TEN) requires immediate transfer to a burn center or intensive care unit with experience in treating these conditions, along with prompt withdrawal of any suspected causative drug.1

Initial Assessment and Triage

1. Immediate actions:

   • Discontinue all potential culprit drugs immediately
   • Calculate SCORTEN within first 24 hours to assess mortality risk
   • Transfer patients with >10% body surface area (BSA) epidermal loss to a burn center or ICU

2. Essential investigations:

   • Full blood count, electrolytes, liver function tests, glucose, magnesium, phosphate
   • Chest X-ray
   • Skin biopsy (from lesional skin adjacent to a blister)
   • Swabs from lesional skin for bacterial culture

Supportive Care

Environment and Nursing

• Barrier-nurse in a side room with controlled humidity
• Use pressure-relieving mattress
• Maintain ambient temperature between 25°C and 28°C 1
• Establish peripheral venous access through non-lesional skin

Skin Management

• Conservative approach:

  • Gently cleanse wounds with warmed sterile water, saline or dilute chlorhexidine
  • Apply greasy emollient (50% white soft paraffin with 50% liquid paraffin) over entire epidermis
  • Leave detached epidermis in situ as a biological dressing
  • Decompress blisters by piercing and draining fluid
  • Apply non-adherent dressings (e.g., Mepitel) to denuded areas
  • Use secondary foam or burn dressings to collect exudate 1

• Monitoring for infection:

  • Take swabs from three areas of lesional skin on alternate days
  • Only administer systemic antibiotics if clinical signs of infection present 1

Mucosal Care

1. Ocular management:

   • Daily ophthalmological review during acute illness
   • Apply preservative-free lubricants every 2 hours
   • Use topical antibiotics if corneal fluorescein staining or ulceration present
   • Prevent corneal exposure in unconscious patients 1

2. Oral care:

   • Daily oral review during acute illness
   • Apply white soft paraffin to lips every 2 hours
   • Clean mouth daily with warm saline mouthwashes
   • Use benzydamine hydrochloride rinse every 3 hours
   • Apply chlorhexidine oral rinse twice daily
   • Use betamethasone sodium phosphate mouthwash four times daily 1

3. Urogenital care:

   • Daily urogenital review during acute illness
   • Apply white soft paraffin to urogenital skin/mucosae every 4 hours
   • Use potent topical corticosteroid ointment on non-eroded surfaces
   • Apply silicone dressings to eroded areas
   • Insert urinary catheter if significant dysuria/retention 1

Respiratory Management

• Monitor for respiratory symptoms and hypoxemia
• If present, arrange urgent discussion with intensivist and transfer to ICU
• Perform fibreoptic bronchoscopy in ICU/burn center setting 1

Systemic Therapy

While the U.K. guidelines do not strongly recommend specific systemic therapies, recent research suggests potential benefits:

• Cyclosporine A has shown increasing evidence of decreasing mortality 2
• Systemic corticosteroids remain one of the most common treatments, though efficacy is uncertain 2
• Combination therapy with corticosteroids and IVIG may warrant further study, as it showed the lowest standardized mortality ratio (0.52) in a large U.S. cohort 3

Discharge and Follow-up

• Provide written information about drugs to avoid
• Encourage wearing a MedicAlert bracelet
• Document drug allergy clearly in patient records
• Report the episode to pharmacovigilance authorities
• Arrange dermatology and ophthalmology follow-up within weeks of discharge 1

Common Pitfalls to Avoid

1. Delayed recognition and transfer - Early recognition and prompt transfer to specialized units is crucial for survival 4

2. Continuing the culprit drug - Immediate withdrawal of suspected causative agents is mandatory 5

3. Inadequate mucosal care - Neglecting daily monitoring of mucosal surfaces can lead to long-term complications including strictures and adhesions 6

4. Inappropriate antibiotic use - Only administer systemic antibiotics when there are clinical signs of infection, not prophylactically 1

5. Overlooking long-term sequelae - Arrange appropriate follow-up with dermatology, ophthalmology, and other specialists to manage potential complications 1