Erythema Multiforme and Related Disorders



Erythema Multiforme and Related Disorders


Julie E. Jackson, MD

Chandra N. Smart, MD








TERMINOLOGY


Abbreviations



  • Erythema multiforme minor (EM)


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


Synonyms



  • Erythema multiforme minor



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


  • Steven-Johnson syndrome/toxic epidermal necrolysis



    • Lyell syndrome


Definitions



  • 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


ETIOLOGY/PATHOGENESIS


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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