Otolaryngology Book

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Erythema Multiforme Major

Aka: Erythema Multiforme Major, Toxic Epidermal Necrolysis, Stevens-Johnson Syndrome, Stevens Johnson
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  1. Definitions
    1. General
      1. Acute life-threatening hypersensitivity skin reaction
    2. Stevens Johnson Syndrome
      1. Affects 10% of body surface area
    3. Toxic Epidermal Necrolysis
      1. Affects 30% of body surface area
  2. Pathophysiology
    1. Severe clinical variant of Erythema Multiforme
    2. Previously thought to be part of same spectrum as minor
      1. Now thought to be clinically distinct
  3. Epidemiology
    1. Most common in winter and early spring
    2. More common in males by 2:1 ratio
    3. Ages affected most commonly 20-40 years
  4. Causes
    1. Medication (50% of Stevens Johnson and 90% of Toxic Epidermal Necrolysis)
      1. Antibiotics (esp. Sulfa drugs)
      2. Seizure medications
      3. NSAIDs
      4. Vaccinations
    2. Other causes
      1. Infectious disease
      2. Connective tissue disease
      3. Pregnancy
  5. Symptoms
    1. Prodrome (onset 1-3 weeks after causative exposure)
      1. Fever
      2. Malaise
      3. Headache
      4. Cough
    2. Sudden widespread skin eruption (onset 1-3 days after exposure)
      1. See below
  6. Signs
    1. Pathognomonic target lesions
      1. Widespread skin involvement
      2. See Erythema Multiforme for course and specific description
    2. Mucosal bullae (severe in 20% of cases)
      1. Oral Mucosa may lead to inadequate oral intake
      2. Respiratory may lead to respiratory distress
      3. Conjunctiva may lead to ocular complications
    3. Lesion distribution
      1. Starts on face and trunk and spreads peripherally
  7. Diagnosis
    1. Clinical diagnosis
    2. Skin biopsy can confirm the diagnosis
  8. Complications
    1. Secondary infection and Sepsis
    2. Multiorgan failure (Renal Failure, Respiratory failure)
    3. Corneal scarring (related to Corneal Ulceration)
  9. Labs
    1. No lab tests are specific for Stevens Johnson syndrome
  10. Management
    1. Intensive care unit (or burn unit) admission
    2. Withdraw all potential causative agents
    3. Fluid Resuscitation
    4. Wound care
    5. Antibiotics only where specifically indicated
    6. IV-Ig has been used in some cases
    7. Ophthalmology consult
  11. Prognosis
    1. Mortality
      1. Stevens Johnson: 5%
      2. Toxic Epidermal Necrolysis: 30%
  12. References
    1. Parrillo (2007) Curr Allergy Asthma Rep 7(4): 243-7
    2. Usatine (2010) Am Fam Physician 82(7): 773-80

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