Updated: 7/9/2020

Erythema Multiforme

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Snapshot
  • A 33-year-old male presents to his physician complaining of several red lesions on his palms, back of hands, and on his lips. His past medical history is significant for recurrent herpes. 
Introduction
  • Hypersensitivity reaction of the skin
  • Common, acute mucocutaneous disease with classic target-shaped lesions with symmetric distribution
  • Divided into erythema multiforme (EM) major and minor
    • major: involves mucous membranes and systemic signs
    • minor: no mucous membrane involvement and no systemic signs
  • Causes
    • most common = infection
      • Herpes simplex virus (most common cause) 
      • Mycoplasma pneumonia
      • upper respiratory infections
    • less common = drugs
      • e.g., sulfonamides, β-lactams, phenytoin
    • idiopathic
  • Often affects young adults (18 - 30 years old especially)
  • Distinct from SJS/TEN, which is a more severe mucocutaneous reaction that is usually caused by a medication
Presentation
  • Skin
    • raised (papular), target lesions with multiple rings and dusky center (as opposed to annular lesions in urticaria)
      • three concentric zones of color from center to outer ring
        • central dusky/dark area that can be crust or vesicle
        • paler pink or edematous zone
        • peripheral red/dark ring
    • fixed lesions (as opposed to urticaria, in which lesions typically resolve within 24 hours)
    • negative Nikolsky sign (as opposed to SJS/TEN)
    • generally mild burning or itchiness; nontender
    • most commonly involves
      • palms/soles
      • backs of hands and feet
      • extensor aspects of forearms and legs
  • Must also examine mucosal surfaces and eyes
    • oral
    • genital/anal
    • iris
  • May have systemic signs (e.g., fever, prodrome)
Evaluation
  • Diagnosis is based on symptoms and clinical history
    • recent history of infection (e.g., herpes or mycoplasma) can aid diagnosis
Differential
  • SJS/TEN (flat lesions, tender, always with mucous involvement, + Nikolsky sign)
  • Urticaria (annular lesions)
  • Erythema nodosum
  • Viral exanthems
Treatment
  • EM major
    • corticosteroids
    • opthalmology consult if ocular involvement
  • EM minor – usually self-limited
    • supportive care
  • Treat underlying cause if identified
    • oral acyclovir for HSV
Prognosis, Prevention, and Complications
  • Prognosis
    • typically self-limited
  • Prevention
    • treat chronic herpes infections
    • if recurrent herpes associated-EM
      • oral acyclovir, valcyclovir, or famciclovir as continuous suppressive therapy
  • Complications
    • usually none associated with EM minor
    • potential ocular complications with EM major
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