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:
- 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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