eMedicine Specialties > Dermatology > Reactive & Inflammatory Dermatoses

Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: Differential Diagnoses & Workup

Author: Peter A Klein, MD, Staff Physician, Department of Dermatology, University Hospital, State University of New York at Stony Brook
Contributor Information and Disclosures

Updated: Feb 20, 2009

Differential Diagnoses

Bullous Pemphigoid
Pemphigus, Paraneoplastic
Burns, Chemical
Psoriasis, Pustular
Graft Versus Host Disease
Staphylococcal Scalded Skin Syndrome
Impetigo
Thermal Burns
Linear IgA Dermatosis
Lupus Erythematosus, Bullous
Pemphigus Vulgaris

Other Problems to Be Considered

Acute generalized exanthematic pustulosis
Linear immunoglobulin A (IgA) bullous dermatosis

Workup

Laboratory Studies

  • The initial laboratory workup includes a CBC count, a chemistry profile, liver enzyme studies, renal function studies, prothrombin time, activated partial thromboplastin time, and cultures of blood and areas of denuded skin.

Imaging Studies

  • A baseline chest radiograph should be obtained because tracheobronchial involvement and respiratory distress are frequent complications.

Procedures

  • Bronchoscopy may be considered to verify involvement of the respiratory tract, but further epithelial trauma may be induced.
  • Similarly, an upper GI series, esophagogastroduodenoscopy, and colonoscopy may be needed to confirm involvement of the GI tract.

Histologic Findings

The biopsy shows a normal stratum corneum with underlying necrosis of epidermal cells (either solitary or en masse). Interface dermatitis with sites of damage out of proportion to the number of lymphocytes is typical.

Diagnosing Stevens-Johnson syndrome/toxic epidermal necrolysis and ruling out staphylococcal scalded skin syndrome or a blistering disorder are important because the prognosis and the course differ markedly. To this end, routine or fresh-frozen section specimens of sloughed epidermis should be obtained for histologic examination. Full-thickness epidermal necrosis is consistent with Stevens-Johnson syndrome/toxic epidermal necrolysis, whereas a subcorneal split is consistent with staphylococcal scalded skin syndrome.

A biopsy sample of fully developed lesions reveals full-thickness epidermal necrosis with involvement of the sweat ducts, relative sparing of the hair follicles, and little alteration of the dermis. Immunofluorescence study results are negative.

More on Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis

Overview: Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis
Differential Diagnoses & Workup: Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis
Treatment & Medication: Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis
Follow-up: Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis
Multimedia: Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis
References
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References

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  2. 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].

  3. Inachi S, Mizutani H, Shimizu M. Epidermal apoptotic cell death in erythema multiforme and Stevens-Johnson syndrome. Contribution of perforin-positive cell infiltration. Arch Dermatol. Jul 1997;133(7):845-9. [Medline].

  4. Viard I, Wehrli P, Bullani R, et al. Inhibition of toxic epidermal necrolysis by blockade of CD95 with human intravenous immunoglobulin. Science. Oct 16 1998;282(5388):490-3. [Medline].

  5. Chan HL, Stern RS, Arndt KA, et al. The incidence of erythema multiforme, Stevens-Johnson syndrome, and toxic epidermal necrolysis. A population-based study with particular reference to reactions caused by drugs among outpatients. Arch Dermatol. Jan 1990;126(1):43-7. [Medline].

  6. Strom BL, Carson JL, Halpern AC, et al. A population-based study of Stevens-Johnson syndrome. Incidence and antecedent drug exposures. Arch Dermatol. Jun 1991;127(6):831-8. [Medline].

  7. Halevy S, Ghislain PD, Mockenhaupt M, Fagot JP, Bouwes Bavinck JN, Sidoroff A. Allopurinol is the most common cause of Stevens-Johnson syndrome and toxic epidermal necrolysis in Europe and Israel. J Am Acad Dermatol. Jan 2008;58(1):25-32. [Medline].

  8. Villada G, Roujeau JC, Cordonnier C, et al. Toxic epidermal necrolysis after bone marrow transplantation: study of nine cases. J Am Acad Dermatol. Nov 1990;23(5 Pt 1):870-5. [Medline].

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  23. Roujeau JC, Kelly JP, Naldi L, et al. Medication use and the risk of Stevens-Johnson syndrome or toxic epidermal necrolysis. N Engl J Med. Dec 14 1995;333(24):1600-7. [Medline].

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Further Reading

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Keywords

Stevens-Johnson syndrome, Stevens Johnson syndrome, toxic epidermal necrolysis, SJS, TEN

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Contributor Information and Disclosures

Author

Peter A Klein, MD, Staff Physician, Department of Dermatology, University Hospital, State University of New York at Stony Brook
Disclosure: Nothing to disclose.

Medical Editor

James J Nordlund, MD, Professor Emeritus, Department of Dermatology, University of Cincinnati College of Medicine
James J Nordlund, MD is a member of the following medical societies: American Academy of Dermatology, Sigma Xi, and Society for Investigative Dermatology
Disclosure: Nothing to disclose.

Pharmacy Editor

David F Butler, MD, Professor of Dermatology, Texas A&M University College of Medicine; Chair, Department of Dermatology, Director, Dermatology Residency Training Program, Scott and White Clinic
David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Association of Military Dermatologists, and Phi Beta Kappa
Disclosure: Nothing to disclose.

Managing Editor

Jeffrey P Callen, MD, Professor of Medicine, Chief, Division of Dermatology, University of Louisville School of Medicine
Jeffrey P Callen, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and American College of Rheumatology
Disclosure: Amgen Honoraria Consulting; Abbott Honoraria Consulting; Electrical Optical Sciences Honoraria Consulting; Centocor Honoraria Consulting; Genetech Honoraria Consulting; Celgene Honoraria Consulting

CME Editor

Joel M Gelfand, MD, MSCE, Medical Director, Clinical Studies Unit, Assistant Professor, Department of Dermatology, Associate Scholar, Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania
Joel M Gelfand, MD, MSCE is a member of the following medical societies: Society for Investigative Dermatology
Disclosure: AMGEN Consulting fee Consulting; AMGEN Grant/research funds None; Genentech Consulting fee Consulting; Centocor Consulting fee Consulting; Centocor Grant/research funds None; Covance Consulting fee Consulting; Shire  Consulting

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

 
 
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