What are the guidelines for diagnosing and treating Stevens-Johnson's syndrome (SJS) and what is the prognosis for patients?

Diagnosis and Management of Stevens-Johnson Syndrome

Patients with Stevens-Johnson syndrome (SJS) with >10% body surface area epidermal loss should be admitted without delay to a burn center or intensive care unit with experience treating SJS/TEN and facilities to manage extensive skin loss wound care. 1

Diagnosis

Clinical Presentation

• Initial "influenza-like" prodromal phase: malaise, fever, anorexia 2
• Painful cutaneous and mucous membrane lesions (ocular, oral, genital) 2
• Classification based on skin detachment:
  • SJS: <10% skin involvement
  • SJS/TEN overlap: 10-30% skin involvement
  • TEN: >30% skin involvement 2

Diagnostic Approach

• Skin biopsy is critical for diagnosis, showing epidermal damage and subepidermal vesicle/bulla formation 3
• Document disease progression with serial assessment and photography 3
• Identify potential causative drugs (high-risk medications include):
  • Anti-infective sulfonamides
  • Anti-epileptic drugs
  • NSAIDs (oxicam type)
  • Allopurinol
  • Nevirapine 2

Initial Management

Immediate Actions

1. Stop the culprit drug immediately 1
2. Transfer to specialized center with burn/ICU capabilities 1
3. Calculate SCORTEN within first 24 hours to predict mortality 1:

Parameter	Points
Age >40 years	1
Heart rate >120 bpm	1
Cancer/hematological malignancy	1
Epidermal detachment >10% BSA	1
Serum urea >10 mmol/L	1
Serum glucose >14 mmol/L	1
Serum bicarbonate <20 mmol/L	1

1. Establish multidisciplinary team coordinated by dermatology/plastic surgery, including intensive care, ophthalmology, and specialist nursing 1

Supportive Care

Environment

• Barrier-nurse in side room with controlled humidity
• Maintain ambient temperature between 25-28°C
• Use pressure-relieving mattress 1

Skin Care

• Minimize shearing forces when handling patient (antishear handling)
• Apply bland emollient to intact skin
• Use appropriate dressings on exposed dermis to reduce fluid/protein loss and limit microbial colonization
• Consider either:
  • Conservative approach: leave detached epidermis in situ
  • Surgical approach: debridement followed by biosynthetic xenograft/allograft (for clinical deterioration) 1

Mucosal Care

Ocular Management:

• Ophthalmological examination within 24 hours 3
• Apply non-preserved lubricants every 2 hours
• Consider moisture chambers for severe cases
• Broad-spectrum topical antibiotic prophylaxis for corneal fluorescein staining/ulceration 1

Oral Management:

• Apply white soft paraffin to lips every 2 hours
• Use mucoprotectant mouthwash three times daily
• Clean mouth daily with warm saline mouthwashes
• Use anti-inflammatory oral rinse/spray (benzydamine hydrochloride) every 3 hours
• Apply antiseptic oral rinse twice daily (hydrogen peroxide or chlorhexidine) 1

Urogenital Management:

• Apply white soft paraffin every 4 hours
• Use Mepitel dressings for eroded areas
• Consider dilator/tampon wrapped in Mepitel for vagina to prevent synechiae
• Catheterize to prevent urethral strictures 1

Respiratory Management

• Monitor for pulmonary complications (can indicate disease severity)
• Be vigilant for bronchial epithelial necrolysis, which may cause sudden airway obstruction 1

Systemic Treatment Options

While no single treatment has proven universally effective, options include:

1. Systemic corticosteroids: Commonly used but efficacy remains uncertain 4
2. Cyclosporine: Increasing evidence shows decreased mortality 4
3. Intravenous immunoglobulin (IVIg): Mixed evidence regarding efficacy 1, 4
4. TNF-α inhibitors: Emerging evidence for decreased mortality 4

Monitoring and Complications

Regular Monitoring

• Daily assessment of vital signs, skin progression, and mucous membranes 3
• Monitor electrolytes, renal and liver function 3
• Vigilance for signs of sepsis (most common cause of death) 1, 3

Common Complications

• Ocular: Sicca syndrome, symblepharon, corneal scarring
• Cutaneous: Scarring, pigmentation changes
• Urogenital: Strictures, synechiae, phimosis
• Respiratory: Bronchiolitis obliterans, bronchiectasis, chronic bronchitis
• Sepsis: Leading cause of morbidity and mortality 2

Prognosis

Mortality rates vary based on SCORTEN score 1, 3:

SCORTEN Points	Predicted Mortality (%)
0	1
1	4
2	12
3	32
4	62
5	85
6	95
7	99

Follow-up Care

• Arrange dermatology follow-up within 4-6 weeks 3
• Provide documentation of causative drug to prevent future exposure
• Consider MedicAlert bracelet 3
• Monitor for long-term sequelae, particularly ocular complications 1

Common Pitfalls to Avoid

• Delayed recognition and transfer to specialized care
• Failure to immediately withdraw culprit drug
• Inadequate mucosal care, particularly ocular management
• Indiscriminate use of antibiotics without signs of infection
• Insufficient pain management
• Neglecting long-term follow-up for complications