How is erythema multiforme (EM) managed in children?

Management of Erythema Multiforme in Children

Erythema multiforme (EM) in children is primarily managed with supportive care alone, as this condition is typically self-limiting with excellent outcomes and rare long-term sequelae. 1, 2

Key Diagnostic Distinction

Before initiating treatment, it is critical to distinguish EM from Stevens-Johnson syndrome (SJS)/toxic epidermal necrolysis (TEN), as these conditions require fundamentally different management approaches 1:

• EM characteristics: Typical target lesions starting on acral surfaces progressing proximally, usually triggered by infection (particularly HSV or Mycoplasma pneumoniae), patients constitutionally well, does not progress to SJS/TEN 1, 2
• EM major: Includes mucosal erosions/ulceration (usually confined to mouth), but patients remain constitutionally well and make good recovery 1
• SJS/TEN: Requires immediate multidisciplinary team referral, potential ICU admission, and specialized wound management 1

Treatment Algorithm

First-Line Management: Supportive Care

Supportive care alone is the primary treatment for pediatric EM, used successfully in 31.1% of reported cases 2:

• Analgesics for pain control 3
• Maintain hydration and nutrition 2
• Gentle wound care for mucosal lesions 3
• Monitor for secondary bacterial infection 2

Second-Line: Topical Therapies

For symptomatic relief of oral lesions 3:

• Topical steroid mouthwash (e.g., dexamethasone or betamethasone rinses) 3
• Topical anesthetics (lidocaine viscous) for oral pain 3
• Vaseline application for lip lesions to prevent adhesion 3

Third-Line: Systemic Corticosteroids (Controversial)

Systemic corticosteroids should NOT be routinely used for pediatric EM, as evidence shows no benefit in recovery time and significant risk of complications 4:

• A retrospective study of 32 pediatric patients with severe EM (Stevens-Johnson syndrome) found that steroid-treated patients did not recover faster than those receiving supportive care only 4
• The steroid-treated group had a significant incidence of medical complications 4
• Despite this, 19.8% of cases in recent literature received systemic corticosteroids, suggesting ongoing practice variation 2

If corticosteroids are considered (against evidence), reserve for severe cases with extensive mucosal involvement where benefits may outweigh risks 2, 4

Management of Specific Triggers

HSV-Associated EM

When HSV is identified as the trigger (17.9% of pediatric cases) 2:

• Antivirals (acyclovir or valacyclovir) were used in 14.6% of cases 2
• Consider for acute episodes with confirmed HSV infection 2, 5

Mycoplasma pneumoniae-Associated EM

When M. pneumoniae is the trigger (15.7% of pediatric cases) 2:

• Macrolide antibiotics (azithromycin, clarithromycin) were used in 7.7% of cases 2
• Treat the underlying respiratory infection 2

Recurrent Erythema Multiforme

Recurrent EM occurs in 14.3% of pediatric cases, with HSV being the trigger in 61% of recurrent cases 2, 5:

Prophylactic Management for Recurrent EM

• Suppressive antiviral therapy (acyclovir or valacyclovir) achieved remission in 31% of patients with recurrent EM 5
• Continuous anti-inflammatory treatment achieved remission in only 25% of patients 5
• Antivirals are preferred over continuous immunosuppression for HSV-associated recurrent EM 5, 3

Special Considerations

Infantile EM (Age <1 year)

• Represents 3.2% of pediatric EM cases 2
• Vaccination is the main trigger (47% of infantile cases) 2
• Infants are significantly less prone to develop EM major compared to older children (p<0.01) 2
• Most cases (89%) are EM minor 2

Age-Related Patterns

• Mean age of onset: 5.6 years (range 0.1-17 years) 2
• Median age for recurrent EM: 9.1 years 5
• Male predominance in recurrent cases (62%) 5

Critical Clinical Pitfalls

Common misdiagnosis: EM is frequently misdiagnosed in emergency departments, with urticaria multiforme being the most common condition incorrectly labeled as EM 6. Only 16% of cases diagnosed as EM in one ED study actually met classification criteria 6.

Lip adhesion: Severe erosive ulceration can cause complete lip adherence, requiring gentle Vaseline application or surgical release under general anesthesia 3.

Avoid ice/water immersion: Unlike erythromelalgia management, EM does not benefit from cooling measures 3.

Prognosis

• Long-term sequelae are rare (1.3% of cases) 2
• EM does not progress to SJS/TEN 1
• Patients typically make good recovery 1, 2
• Hospitalization required in 35% of recurrent EM cases 5