ERYTHEMA MUTIFORME MINOR

 

ERYTHEMA MUTIFORME MINOR

 

DEFINITION:

An acute mucocutaneous hypersensitivity reaction resulting in the formation of symmetric, discrete, red papules and plaques of variable morphology which tend to recur.

EPIDEMIOLOGY:

  • incidence: 1/1,000-10,000
  • age of onset:
    • peaks between 20-40 years of age
    • 20% of cases occur in children
  • risk factors:
    • exposure to triggers (see below)
    • winter, early spring

PATHOGENESIS:

1. Background

1. Forms of Erythema Multiforme (EM)

  • 1. Minor - Classic EM
  • 2. Major - Stevens-Johnson Syndrome; Toxic Epidermal Necrolysis
  • 2. Triggers

  • 1. Drugs
    • allopurinol
    • anticonvulsants
    • barbiturates
    • sulfonamide antibiotics
  • 2. Infections
    • bacterial - diphtheria, mycoplasma, syphilis, TB, typhoid, yersinia, group A streptococcus
    • viral - EBV, enteroviruses, HSV, mumps, polio, vaccinia
    • fungal - coccidiomycosis, histoplasmosis
  • 3. Immune Diseases
    • Graft vs Host Disease
    • Inflammatory Bowel Disease
    • Polyarteritis Nodosa
    • SLE
  • 4. Others
    • chemicals - nitrobenzene, perfumes, terpenes
    • immunizations - BCG, hepatitis B
    • malignancy - leukemia, lymphoma
    • radiotherapy
    • Sarcoidosis
  • 3. Pathogenesis

    • exposure to trigger -> host immune response to a foreign antigen -> EM
    • HSV-induced EM:
      • accounts for 15-63% of cases of EM Minor
      • associated with recurrent HSV and occurs about 10 days following the onset of HSV; associated with each outbreak of HSV
      • not associated with a primary HSV infection

    CLINICAL FEATURES:

    1. Prodrome

    • occurs in about 30% of cases
    • flu-like symptoms:
      • sore throat, headache, arthralgias, myalgias, fever
    • lasts 1-2 weeks before the onset of the skin manifestations in 33% of patients
    • may begin within 1-3 weeks after starting a new drug

    2. Skin Manifestations

    1. Multiforme

    1. Background

    • acute onset of rash which is self-limiting
    • represents the evolution of fixed skin lesions through multiple morphologic stages and not the occurence of several different types of skin lesions
    • evolves from macules to papules to plaques over 3-5 days rather than over hours
    • skin lesions may vary between patients, within a patient, or between episodes
    • location:
      • commonly affects the distal extremities involving the extensor surfaces of the arms, legs, elbows, knees, dorsum of hands and feet, and nailfolds
      • areas usually spared include the scalp, face, neck, trunk, perineum, palms, and soles
      • tends to spread from the extensor surfaces of the extremities to the flexural surfaces and then to the trunk
      • propensity for areas exposed to light and/or physical trauma

    2. Rash

  • 1. Macules
    • symmetric, round, and erythematous
    • 1 cm to several cm's in diameter
    • slightly pruritic or non-itchy; may also burn
    • evolve into papules or plaques over days
  • 2. Papules/Plaques
    • frequently display a circumferential pallor
    • evolve into target or iris lesions
    • target lesions:
      • large annular (ring-shaped) lesions with concentric colour changes
      • round and well demarcated
      • centre of the papule forms a necrotic ulceration which results in a depressed white, yellow, or grey area
      • surrounding this central region is the red edge of the papule and then a pale edematous ring; a bright red margin may surround this pale ring
      • further evolution of the central region results in central clearing, crusting, or the formation of small vesicles or bullae
      • lesions may recur in crops for 2-4 weeks
      • resolution occurs with crusting or scaling of the target lesions
      • individual lesions heal in 1-4 weeks (rather than in months)
      • usually no scar formation but post-inflammatory hypo- or hyperpigmentation may occur
    • recurrence rate of 25% especially with HSV-induced EM
  • 2. Others

    • mild cutaneous blistering may occur
    • edema
    • Koebners phenomenon

    3. Mucous Membrane Manifestations

    • occur in about 60% of cases
    • mild involvement usually restricted to the mouth:
      • erythema and edema
      • aphthous-like shallow oral ulcerations
    • may occur simultaneously with, precede, or follow the cutaneous manifestations
    • there may also be mild conjunctival injection

    INVESTIGATIONS:

    1. Acute Phase Reactants

    • elevated ESR, WBC, platelets, circulating immune complexes

    2. Serology

    • for various bacteria and viruses

    3. Skin Biopsy

  • 1. Small Macules and Papules:
    • damage to the dermis
      • perivascular mononuclear inflammation along with deposition of immunoglobulins and complement
      • cytotoxic T cells
      • mild dermal edema -> large subepidermal blisters
  • 2. Large Papules or Plaques and Target Lesions:
    • damage to the epidermis
      • necrosis of individual basal cell keratinocytes or throughout the entire epidermis
  • MANAGEMENT:

    1. Supportive

  • 1. Prophylactic
    • avoid triggers
    • adequate hydration and nutritional support
  • 2. Pruritis
    • warm water tub soaks
    • benadryl or atarax
  • 3. Oral Lesions
    • pink lady (Diovol 20cc + 2% Viscous Xylocaine 5cc)
  • 2. Medical

  • 1. Prednisone
    • indication: recurrent EM
    • dose: 0.5-1.0 mg/kg/day tapered over 7-10 days
    • contraindication: HSV-induced EM
  • 2. Acyclovir
    • indication: HSV-induced EM
    • 5 day course at the first sign of lesions
  • REFERENCES:

  • 1. Contemporary Pediatrics 11:19 (1994)
  • 2. Pediatric Clinics of North America
  • 3. Nelson Textbook of Pediatrics (1992) p. 1639-1642
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    Pediatric Database - ERYTHEMA MUTIFORME MINOR

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