Can Herpes Simplex Virus (HSV) cause Stevens-Johnson Syndrome (SJS)?

Herpes Simplex Virus as a Cause of Stevens-Johnson Syndrome

Yes, herpes simplex virus (HSV) can cause Stevens-Johnson Syndrome (SJS), particularly in children, and is recognized as an important infectious trigger of this severe mucocutaneous reaction. 1

Relationship Between HSV and SJS

Infectious Triggers of SJS

• HSV is specifically identified in multiple guidelines as a known infectious trigger for SJS/TEN 1
• While medications are the most common cause of SJS/TEN in adults, infections are more frequently implicated in children, with up to 50% of pediatric cases attributed to infectious causes 1
• HSV joins Mycoplasma pneumoniae as one of the most frequently documented infectious causes of SJS 2, 3

Clinical Distinction

• It's important to distinguish between:
  • Erythema multiforme major (EMM): Typically caused by HSV and predominantly affects the extremities with typical target lesions 1
  • SJS: More severe reaction with widespread purpuric macules or flat atypical targets and less than 10% body surface area (BSA) involvement 1
  • SJS/TEN overlap: 10-30% BSA involvement 1
  • TEN: Greater than 30% BSA involvement 1

Diagnostic Considerations

Clinical Features

• SJS presents with:
  • Widespread purpuric macules or flat atypical targets
  • Mucosal involvement (often severe)
  • Epidermal detachment (<10% BSA in SJS)
  • Prodromal symptoms including fever, malaise, and respiratory symptoms 1

Histopathology

• Skin biopsy shows:
  • Variable epidermal damage ranging from individual cell apoptosis to confluent epidermal necrosis
  • Basal cell vacuolar degeneration
  • Subepidermal vesicle or bulla formation
  • Mild perivascular infiltrate of lymphocytes and histiocytes 1

Testing for HSV

• When HSV is suspected as a trigger:
  • PCR testing of vesicular fluid
  • Serological testing for HSV antibodies
  • Testing for other potential infectious triggers (particularly M. pneumoniae) should be performed concurrently 1

Management of HSV-Associated SJS

Immediate Actions

• Prompt recognition and early intervention are critical 2
• Discontinue any potential triggering medications 1
• Initiate antiviral therapy (acyclovir) when HSV is identified or strongly suspected 4, 5

Treatment Approach

• Supportive care remains the cornerstone of management:
  • Specialized care in appropriate units (burn centers or intensive care units for severe cases)
  • Wound care and prevention of secondary infections
  • Fluid and electrolyte management
  • Nutritional support
  • Ophthalmological care for ocular involvement 1, 2

Prevention of Recurrence

• HSV-associated SJS has been documented to recur in some patients 4, 5
• Prophylactic strategies for patients with recurrent HSV-associated SJS include:
  • Continuous low-dose acyclovir prophylaxis
  • Early intervention with higher-dose acyclovir and prednisone at the first sign of HSV reactivation 4, 5, 6
  • This approach has been shown to successfully prevent progression to SJS in patients with recurrent disease 5

Special Considerations in Children

• SJS recurrence is more common in children (up to 18% of cases) 2
• Children with HSV-associated SJS should be managed in age-appropriate specialist units 1
• Prophylactic antiviral therapy may be considered in children with recurrent HSV-associated SJS 2

Prognosis and Complications

• Mortality rates vary: 1-5% for SJS, 25-35% for TEN 3
• Long-term sequelae may include:
  • Skin pigmentation changes
  • Scarring
  • Ocular complications
  • Respiratory and urogenital problems 2, 3

HSV-associated SJS requires prompt recognition, immediate treatment with antivirals when indicated, and comprehensive supportive care to minimize morbidity and mortality. For patients with recurrent HSV-associated SJS, prophylactic strategies should be strongly considered to prevent future episodes.