What is the treatment for Stevens-Johnson syndrome?

Treatment of Stevens-Johnson Syndrome

The treatment of Stevens-Johnson Syndrome (SJS) requires immediate transfer to a burn center or intensive care unit, prompt withdrawal of any suspected causative drug, and implementation of supportive care measures including fluid resuscitation, wound care, and management of mucosal involvement. 1

Initial Management

Immediate Actions

• Transfer to specialized center for patients with:
  • Confirmed TEN (>30% skin detachment)
  • SJS/TEN overlap with poor prognostic factors
  • Severe eye disease requiring specialist services 2
• Calculate SCORTEN within first 24 hours to assess mortality risk 1
• Discontinue all suspected causative medications immediately 2, 1
• Maintain ambient temperature between 25-28°C 1

Fluid Management

• Establish adequate intravenous fluid replacement through non-lesional skin 2
• Monitor fluid balance carefully and catheterize if clinically indicated 2
• Use continuous invasive hemodynamic monitoring in severe cases 2
• Be cautious of overhydration and resultant hyponatremia 2

Skin Care

Conservative Approach

• Gently cleanse wounds using warmed sterile water, saline, or dilute chlorhexidine (1/5000) 2
• Apply greasy emollient (50% white soft paraffin with 50% liquid paraffin) over the entire epidermis 2
• Leave detached epidermis in situ as a biological dressing 2, 1
• Decompress blisters by piercing and draining fluid 2, 1
• Apply non-adherent dressings to denuded dermis (Mepitel™ or Telfa™) 2
• Use secondary foam or burn dressing to collect exudate 2
• Handle skin carefully to minimize epidermal detachment 2

Infection Prevention

• Take swabs for bacterial and candidal culture from lesional skin throughout acute phase 2
• Apply topical antimicrobial agents only to sloughy areas 2
• Consider silver-containing products/dressings (limited use if extensive areas) 2
• Do not administer prophylactic systemic antibiotics - only treat when clinical signs of infection are present 1, 2

Mucosal Care

Ocular Management

• Perform daily ophthalmological review during acute illness 1
• Apply preservative-free lubricants every 2 hours 1
• Use topical antibiotics if corneal fluorescein staining or ulceration present 2
• Consider topical corticosteroid drops (nonpreserved dexamethasone 0.1%) under ophthalmologist supervision 2
• Prevent corneal exposure in unconscious patients using moisture chamber with polyethylene film 2

Oral Care

• Apply white soft paraffin to lips every 2 hours 1
• Clean mouth daily with warm saline mouthwashes 2, 1
• Use benzydamine hydrochloride rinse every 3 hours, particularly before eating 2, 1
• Consider topical anesthetic preparations (viscous lidocaine 2%) for severe pain 2
• Use antiseptic oral rinse twice daily (hydrogen peroxide 1.5% or chlorhexidine 0.2%) 2
• Consider topical corticosteroids four times daily for inflammation 2

Urogenital Care

• Examine urogenital tract as part of initial assessment 2
• Apply white soft paraffin to urogenital skin/mucosae every 4 hours 2, 1
• Use silicone dressings (Mepitel) on eroded areas to reduce pain and prevent adhesions 2, 1
• Consider potent topical corticosteroid ointment on non-eroded surfaces 2, 1
• Catheterize patients to prevent urethral strictures 2

Systemic Therapy

Corticosteroids

• For immune checkpoint inhibitor-induced SJS/TEN:
  • Administer IV methylprednisolone 0.5-1 mg/kg for Grade 3
  • Increase to 1-2 mg/kg for Grade 4 2
  • Convert to oral corticosteroids on response, wean over at least 4 weeks 2
• For conventional SJS/TEN, corticosteroid use remains controversial but is commonly used 3, 4

Other Immunomodulatory Therapies

• Intravenous immunoglobulin (IVIG) or cyclosporine may be considered in severe or steroid-unresponsive cases 2, 5, 4
• Cyclosporine and TNF-α inhibitors have shown promise in decreasing mortality 4

Pain Management

• Use appropriate validated pain assessment tools at least once daily 2
• Administer adequate analgesia using intravenous opioid infusions if oral medication not tolerated 2
• Consider patient-controlled analgesia where appropriate 2
• Consider sedation or general analgesia for painful procedures 2

Follow-up Care

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

Cautions and Pitfalls

• Do not delay transfer to specialized centers - early referral is associated with better outcomes 5
• Avoid indiscriminate use of prophylactic antibiotics as this may increase skin colonization 2
• Be vigilant for sepsis which may be masked by disease-associated fever 2
• Avoid adhesive dressings, ECG leads, and identification wrist tags that can cause further skin trauma 2
• Do not overlook long-term sequelae - arrange appropriate specialist follow-up 1