eMedicine Specialties > Infectious Diseases > Skin and Soft-Tissue Infections

Erythema Multiforme (Stevens-Johnson Syndrome): Differential Diagnoses & Workup

Author: Don R Revis Jr, MD, Consulting Staff, Department of Surgery, Division of Plastic and Reconstructive Surgery, University of Florida College of Medicine
Contributor Information and Disclosures

Updated: Aug 5, 2009

Differential Diagnoses

Other Problems to Be Considered

Acute generalized exanthematous pustulosis
Bullous pemphigoid
Bullous phototoxic reactions
Chemical burns
Erythroderma
Exfoliative dermatitis
Immunoglobulin A (IgA) linear dermatosis
Maculopapular drug rashes
Paraneoplastic pemphigus acantholysis
Pemphigus vulgaris
Staphylococcal scalded skin syndrome
Thermal burns
Toxic epidermal necrolysis (TEN)
Lyme disease

Workup

Laboratory Studies

  • No laboratory tests are specific to Stevens-Johnson syndrome (SJS).
  • The following laboratory abnormalities are frequently encountered:  
    • Lymphopenia, possibly secondary to the depletion of CD4 lymphocytes (90% of patients)
    • Neutropenia (30% of patients), which indicates a poor prognosis
    • Thrombocytopenia (15% of patients)
    • Anemia
    • Prerenal azotemia and elevated serum urea nitrogen levels (indicate a poor prognosis)
    • Elevated erythrocyte sedimentation rate
    • Elevated serum aminotransferase levels

Other Tests

  • Direct immunofluorescence staining and examination may identify an alternative diagnosis (eg, pemphigoid, IgA linear dermatosis).

Procedures

  • Perform a skin biopsy of the cutaneous lesions to confirm or exclude the presence of other blistering disorders.

Histologic Findings

  • Initially, vacuolar change occurs at the dermoepidermal junction and shows sparse lymphocytic and macrophage infiltration.
  • This vacuolar change represents individual or small groups of necrotic (apoptotic) keratinocytes.
  • Vacuolization then becomes confluent, which is clinically observed as blistering.
  • The overlying epidermis shows full-thickness necrosis and sloughs off at the dermoepidermal junction, exposing a relatively normal-appearing dermis.
  • The upper dermis displays mild inflammation with perivascular lymphohistiocytic infiltrates.
  • Immunohistopathological analysis shows a predominance of CD8 T cells and macrophages in the epidermis, whereas CD4 T cells form the perivascular infiltrates in the papillary dermis.
  • Histological examination of skin biopsies in staphylococcal scalded skin syndrome reveals cleavage of cell layers within the epidermis.

More on Erythema Multiforme (Stevens-Johnson Syndrome)

Overview: Erythema Multiforme (Stevens-Johnson Syndrome)
Differential Diagnoses & Workup: Erythema Multiforme (Stevens-Johnson Syndrome)
Treatment & Medication: Erythema Multiforme (Stevens-Johnson Syndrome)
Follow-up: Erythema Multiforme (Stevens-Johnson Syndrome)
Multimedia: Erythema Multiforme (Stevens-Johnson Syndrome)
References
Further Reading
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References

  1. von Hebra F. Acute exantheme und hautkrankheiten. Handbuch der Speciellen Pathologie und Therapie. 1860;198-200.

  2. Stevens AM, Johnson FC. A new eruptive fever associated with stomatitis and ophthalmia: report of two cases in children. Am J Dis Child. 1922;24:526-33.

  3. Lyell A. Toxic epidermal necrolysis: an eruption resembling scalding of the skin. Br J Dermatol. Nov 1956;68(11):355-61. [Medline].

  4. Tan YM, Goh KL. Esophageal stricture as a late complication of Stevens-Johnson syndrome. Gastrointest Endosc. Oct 1999;50(4):566-8. [Medline].

  5. Bastuji-Garin S, Rzany B, Stern RS, Shear NH, Naldi L, Roujeau JC. Clinical classification of cases of toxic epidermal necrolysis, Stevens-Johnson syndrome, and erythema multiforme. Arch Dermatol. Jan 1993;129(1):92-6. [Medline].

  6. Guitart J. Immunopathology of Stevens-Johnson syndrome. Allergy Proc. Jul-Aug 1995;16(4):163-4. [Medline].

  7. Knowles S, Shapiro L, Shear NH. Serious dermatologic reactions in children. Curr Opin Pediatr. Aug 1997;9(4):388-95. [Medline].

  8. Lehman SS. Long-term ocular complication of Stevens-Johnson syndrome. Clin Pediatr (Phila). Jul 1999;38(7):425-7. [Medline].

  9. Manders SM. Serious and life-threatening drug eruptions. Am Fam Physician. Jun 1995;51(8):1865-72. [Medline].

  10. Mockenhaupt M, Schopf E. Epidemiology of drug-induced severe skin reactions. Semin Cutan Med Surg. Dec 1996;15(4):236-43. [Medline].

  11. Posadas SJ, Leyva L, Torres MJ, Rodriguez JL, Bravo I, Rosal M, et al. Subjects with allergic reactions to drugs show in vivo polarized patterns of cytokine expression depending on the chronology of the clinical reaction. J Allergy Clin Immunol. Oct 2000;106(4):769-76. [Medline].

  12. Revuz JE, Roujeau JC. Advances in toxic epidermal necrolysis. Semin Cutan Med Surg. Dec 1996;15(4):258-66. [Medline].

  13. Roujeau JC. Severe drug-induced blistering disorders. Rev Rhum Engl Ed. Jan 1997;64(1):5-9. [Medline].

  14. Roujeau JC. Stevens-Johnson syndrome and toxic epidermal necrolysis are severity variants of the same disease which differs from erythema multiforme. J Dermatol. Nov 1997;24(11):726-9. [Medline].

  15. Roujeau JC. The spectrum of Stevens-Johnson syndrome and toxic epidermal necrolysis: a clinical classification. J Invest Dermatol. Jun 1994;102(6):28S-30S. [Medline].

  16. Roujeau JC, Stern RS. Severe adverse cutaneous reactions to drugs. N Engl J Med. Nov 10 1994;331(19):1272-85. [Medline].

  17. Weston WL. What is erythema multiforme?. Pediatr Ann. Feb 1996;25(2):106-9. [Medline].

  18. Wolkenstein P, Revuz J. Drug-induced severe skin reactions. Incidence, management and prevention. Drug Saf. Jul 1995;13(1):56-68. [Medline].

  19. Wong KC, Kennedy PJ, Lee S. Clinical manifestations and outcomes in 17 cases of Stevens-Johnson syndrome and toxic epidermal necrolysis. Australas J Dermatol. Aug 1999;40(3):131-4. [Medline].

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Keywords

erythema multiforme, Stevens-Johnson syndrome, erythema multiforme major, erythema multiforme bullosum, bullous erythema multiforme, erythema multiform exudativum, SJS, toxic epidermal necrolysis, TEN, SJS/TEN overlap, Stevens-Johnson syndrome/toxic epidermal necrolysis overlap, target lesions, iris lesions, herpes simplex virus, HSV, Epstein-Barr virus, histoplasmosis, keratinocyte necrosis, febrile erosive stomatitis, severe conjunctivitis, disseminated cutaneous eruption, erythema multiforme minor

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Contributor Information and Disclosures

Author

Don R Revis Jr, MD, Consulting Staff, Department of Surgery, Division of Plastic and Reconstructive Surgery, University of Florida College of Medicine
Don R Revis Jr, MD is a member of the following medical societies: American College of Surgeons, American Medical Association, American Society for Aesthetic Plastic Surgery, and American Society of Plastic Surgeons
Disclosure: Nothing to disclose.

Medical Editor

Jeffrey Lee Kishiyama, MD, Assistant Clinical Professor of Medicine, University of California at San Francisco School of Medicine; Consulting Staff, Allergy and Asthma Associates of Santa Clara Valley Research Center
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Michael R Simon, MD, MA, Clinical Professor Emeritus, Departments of Internal Medicine and Pediatrics, Wayne State University School of Medicine; Adjunct Staff, Division of Allergy and Immunology, Department of Internal Medicine, William Beaumont Hospital
Michael R Simon, MD, MA is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American College of Allergy, Asthma and Immunology, American College of Physicians, American Federation for Medical Research, Michigan Allergy and Asthma Society, Michigan State Medical Society, Royal College of Physicians and Surgeons of Canada, and Society for Experimental Biology and Medicine
Disclosure: Secretory IgA, Inc. Ownership interest Board membership

CME Editor

Timothy D Rice, MD, Associate Professor, Departments of Internal Medicine and Pediatrics and Adolescent Medicine, Saint Louis University School of Medicine
Timothy D Rice, MD is a member of the following medical societies: American Academy of Pediatrics and American College of Physicians
Disclosure: Nothing to disclose.

Chief Editor

Burke A Cunha, MD, Professor of Medicine, State University of New York School of Medicine at Stony Brook; Chief, Infectious Disease Division, Winthrop-University Hospital
Burke A Cunha, MD is a member of the following medical societies: American College of Chest Physicians, American College of Physicians, and Infectious Diseases Society of America
Disclosure: Nothing to disclose.

 
 
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