Erythema Multiforme (EM)

 

Epidemiology:

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Common sites:

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Gross features:

    • symmetric involvement of the extremities frequently
    • multiform lesions
      • macules
      • papules
      • vesicles
      • bullae
    • characteristic target lesion
      • red macule or papule with a pale, vesicular, or eroded centre
    • Stevens-Johnson syndrome:
      • Erosions and hemorrhagic crusts involving lips and oral mucosa
        • conjunctiva, urethra, genital, and perianal areas may be involved
    • toxic epidermal necrolysis (TEN):
      • diffuse necrosis and sloughing of cutaneous and mucosal epithelial surfaces

 

Histologic features:

    • prototypical cytotoxic reaction pattern
    • early:
      • superficial perivascular, lymphocytic infiltrate
      • dermal edema
      • interface dermatitis
        • accumulation of lymphocytes along dermoepidermal junction
        • intimately associated with degenerating and necrotic keratinocytes
        • basal cell vacuolation
        • CD8+ cytotoxic T cells kill the epithelial cells
    • later:
      • upward migration of lymphocytes into epidermis
      • discrete and confluent zones of epidermal necrosis with blister formation and erosions

 

Immunophenotype:

Marker:

Sensitivity:

Specificity:

 

 

 

 

Molecular features:

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Other features:

    • hypersensitivity reaction to certain infections and drugs
      • infections:
        • herpes simplex
        • mycoplasmal infections
        • histoplasmosis
        • coccidioidomycosis
        • typhoid
        • leprosy
        • others
      • drugs:
        • sulfonamides
        • penicillin
        • barbiturates
        • salicylates
        • hydantoins
        • antimalarials
      • malignancies:
        • carcinomas
        • lymphomas
      • collagen vascular diseases:
        • lupus erythematosus
        • dermatomyositis
        • periarteritis nodosa
    • Stevens-Johnson syndrome is an extensive and symptomatic febrile form:
      • Often children
      • May have superinfection and sepsis
    • Toxic epidermal necrolysis is another variant

 

References:

    • Robbins 2005