Erythema Multiforme and Related Disorders

Erythema Multiforme and Related Disorders

Julie E. Jackson, MD

Chandra N. Smart, MD



  • Erythema multiforme minor (EM)

  • Steven-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN)


  • Erythema multiforme minor

    • Erythema multiforme von Hebra, herpes simplex-associated erythema multiforme

  • Steven-Johnson syndrome/toxic epidermal necrolysis

    • Lyell syndrome


  • EM minor (von Hebra): Acute, self-limited, immune-mediated reaction to infections or drugs consisting of typical targetoid skin lesions with mild or no mucous membrane involvement

  • SJS/TEN: Serious, life-threatening reaction often to medications consisting of atypical targetoid lesions that evolve to extensive epidermal necrosis and epidermal sloughing with severe mucous membrane involvement and systemic symptoms

    • SJS: 10% of total body surface area affected

    • TEN: 30% of total body surface affected


Infectious Agents (Major Causes of EM Minor)

  • Herpes simplex virus types 1 and 2

  • Mycoplasma pneumoniae

Drug Mediated (Major Causes of SJS/TEN)

  • Sulfonamides

  • Antibiotics

    • Aminopenicillins, cephalosporins, quinolones

  • Anticonvulsants

    • Phenytoin, barbiturates, carbamazepine

  • Nonsteroidal anti-inflammatory medications

    • Phenylbutazone, oxyphenbutazone, isoxicam, piroxicam

Other Associations

  • Environmental factors, radiocontrast media, connective tissue disease, neoplasms, pregnancy, vaccinations

Pathogenesis of SJS/TEN

  • Fas ligand-mediated apoptosis is probably central to development of epidermal necrosis

Jul 8, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Erythema Multiforme and Related Disorders

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