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

Immediate Management of Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis

Patients with Stevens-Johnson Syndrome (SJS) or Toxic Epidermal Necrolysis (TEN) must be immediately admitted to a burn center or intensive care unit with experience in managing these conditions, with prompt discontinuation of the culprit drug as the first critical step in management. 1

Initial Assessment and Triage

• Calculate SCORTEN within the first 24 hours to predict mortality risk 2
• Document extent of skin involvement (% body surface area affected) using body mapping 2
• Perform full physical examination to assess:
  • Vital signs and oxygen saturation
  • Airway patency (involve anesthesia immediately if concerns)
  • Skin for target lesions, purpuric macules, blisters, and epidermal detachment
  • Mucosal involvement (mouth, eyes, genitalia) 2
• Obtain skin biopsy if diagnostic uncertainty exists 2

Immediate Management Steps

1. Transfer to specialized unit:

   • Admit to burn center or ICU if >10% body surface area involvement 2, 1
   • Barrier-nurse in a side room with controlled humidity
   • Place on pressure-relieving mattress
   • Maintain ambient temperature between 25-28°C 2, 1

2. Discontinue potential culprit medications:

   • Immediately stop all suspected causative drugs 2, 1
   • Document all medications taken in previous 2 months 2

3. Supportive care:

   • Fluid management: Provide adequate IV fluid replacement through non-lesional skin
   • Monitor fluid balance carefully (requirements are lower than predicted by burn formulas) 1
   • Pain control: Follow WHO analgesic ladder principles; consider opiates for moderate-to-severe pain 1

4. Wound care:

   • Handle skin carefully to minimize shearing forces
   • Gently cleanse wounds with warmed sterile water, saline, or dilute chlorhexidine
   • Apply greasy emollients (50% white soft paraffin with 50% liquid paraffin) over intact epidermis
   • Leave detached epidermis in situ as biological dressing
   • Apply non-adherent dressings to denuded areas 1
   • Avoid aggressive debridement initially 1

5. Specialized care for affected areas:

   • Eyes: Arrange ophthalmology examination within 24 hours 2, 1

     • Apply preservative-free lubricants every 2 hours
     • Consider topical antibiotics and corticosteroid drops under ophthalmologist supervision
     • Use moisture chambers for unconscious patients

   • Oral mucosa:

     • Clean mouth daily with warm saline mouthwashes
     • Consider topical corticosteroids (betamethasone sodium phosphate)
     • Use benzydamine hydrochloride rinse every 3 hours 1

   • Urogenital tract:

     • Apply white soft paraffin to urogenital skin/mucosae every 4 hours
     • Use silicone dressings on eroded areas
     • Consider catheterization to prevent urethral strictures 1

6. Infection prevention:

   • Monitor for sepsis (may be masked by disease-associated fever)
   • Obtain bacterial swabs from lesional skin for culture
   • Avoid prophylactic antibiotics without clinical signs of infection 2, 1
   • Consider prophylactic anticoagulation for immobile patients 1

Systemic Therapy Options

For severe cases (≥10% BSA involvement):

• Administer IV methylprednisolone 1-2 mg/kg, tapering when toxicity resolves 2, 1
• Consider IVIG or cyclosporine for severe or steroid-unresponsive cases 2, 1

For less severe cases:

• IV methylprednisolone (or equivalent) 0.5-1 mg/kg, converting to oral corticosteroids on response, with taper over at least 4 weeks 2, 1

Multidisciplinary Approach

Coordinate care through a multidisciplinary team including:

• Dermatology and/or plastic surgery (lead)
• Intensive care
• Ophthalmology
• Specialist skincare nursing
• Additional specialists as needed (respiratory, gastroenterology, gynecology, urology, etc.) 2, 1

Investigations

Order the following tests:

• Full blood count, C-reactive protein, urea and electrolytes
• Liver function tests and coagulation studies
• Glucose, magnesium, phosphate, bicarbonate, base excess, lactate
• Infection screening (mycoplasma, chlamydia, HSV)
• Bacterial swabs from lesional skin 2

Pitfalls to Avoid

• Delayed transfer to specialized unit increases mortality 2
• Prophylactic antibiotics may increase skin colonization with resistant organisms 1
• Aggressive debridement can worsen skin damage 1
• Failure to involve ophthalmology early can lead to permanent ocular complications 2, 1