What are the warning signs and management of Stevens-Johnson Syndrome (SJS) after an allergic reaction to medication?

Warning Signs and Management of Stevens-Johnson Syndrome After Allergic Reaction to Medication

Immediate discontinuation of the suspected culprit medication is the most critical first step in managing Stevens-Johnson Syndrome (SJS), followed by urgent referral to a specialized burn unit or dermatology service for comprehensive care. 1

Warning Signs of SJS

Early recognition of SJS warning signs is crucial for prompt intervention:

Prodromal Phase (1-3 days before skin manifestations)

• Fever (often >39°C)
• Flu-like symptoms (malaise, fatigue)
• Upper respiratory symptoms
• Sore throat and eyes
• Burning sensation in eyes
• General discomfort

Early Cutaneous and Mucosal Signs

• Painful skin (rather than itchy)
• Erythematous macules that rapidly evolve
• Target-like lesions or atypical targets
• Mucosal involvement (often precedes skin lesions):
  • Painful erosions in mouth/lips
  • Conjunctival redness, burning, discharge
  • Genital/urethral pain or erosions
• Nikolsky sign (skin sloughing with lateral pressure)

Alarming Signs Requiring Immediate Medical Attention

• Any mucosal involvement plus skin rash after medication exposure
• Blisters or skin detachment of any percentage
• Purpuric macules
• Facial swelling with mucosal symptoms
• Difficulty swallowing or breathing
• Skin pain out of proportion to appearance 1, 2

Initial Management Steps

1. Immediately discontinue all potential culprit medications 1
2. Urgent referral to emergency department or specialized unit (burn center or dermatology service) 1, 3
3. Complete assessment:
   • Document all medications taken in previous 2 months
   • Create timeline of drug exposure (5-28 days prior is highest risk period)
   • Record extent of skin involvement using body map
   • Examine all mucosal sites
   • Calculate SCORTEN score for mortality prediction 1, 3

Diagnostic Workup

• Full blood count, ESR, CRP, renal and liver function tests
• Skin biopsy from lesional skin adjacent to blister
• Chest X-ray
• Swabs from lesional skin for bacteriology
• Clinical photographs to document progression 1

Hospital Management

Supportive Care

• Transfer to burn unit or ICU with experience in SJS/TEN
• Maintain ambient temperature 25-28°C
• Barrier nursing in side room with controlled humidity
• Pressure-relieving mattress to prevent further skin damage
• Fluid resuscitation based on extent of skin involvement
• Nutritional support (nasogastric if needed) 1, 3

Wound Care

• Apply emollients to intact skin
• Use non-adherent dressings (Mepitel or Telfa) on denuded areas
• Gentle cleaning with sterile water or saline
• Consider antimicrobial agents such as chlorhexidine (1/5000) 3

Specialized Care for Affected Areas

• Eyes: Non-preserved lubricants every 2 hours, consider moisture chambers, topical antibiotic prophylaxis
• Mouth: White soft paraffin to lips every 2 hours, mucoprotectant mouthwash
• Genitals: White soft paraffin every 4 hours, consider dilator/tampon wrapped in Mepitel for vagina 3

Monitoring

• Daily assessment for signs of sepsis (most common cause of death)
• Regular monitoring of electrolytes, renal and liver function
• Vigilance for pulmonary complications 3

Treatment Considerations

The optimal treatment remains controversial, but options include:

• Systemic corticosteroids: IV methylprednisolone 0.5-1 mg/kg/day with tapering over at least 4 weeks 1
• Intravenous immunoglobulin (IVIg): Evidence is mixed but may be considered
• Cyclosporine A: Has shown promising results in some studies 2

Long-term Follow-up

• Arrange ophthalmology follow-up within weeks of discharge if eye involvement occurred
• Dermatology follow-up within 4-6 weeks
• Document causative drug and provide patient with information
• Consider MedicAlert bracelet/amulet
• Report to pharmacovigilance authorities 1, 3

Prognosis

Mortality depends on SCORTEN score and ranges from 1% (score 0) to 90% (score ≥5). Long-term sequelae are common, particularly ocular complications, which can be severe and require ongoing management 3, 4.

Common Culprit Medications

High-risk medications for SJS/TEN include:

• Anti-epileptic drugs (carbamazepine, phenytoin, phenobarbital)
• Antibiotics (sulfonamides, aminopenicillins, quinolones)
• Allopurinol
• NSAIDs (particularly oxicam type)
• Nevirapine 2, 1

Early recognition of warning signs and prompt management are critical for improving outcomes in this potentially life-threatening condition.