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Toxic Epidermal Necrolysis: Differential Diagnoses & Workup

Author: Gregory P Garra, DO, Clinical Assistant Professor, Department of Emergency Medicine, Stony Brook University School of Medicine; Residency Program Director, Department of Emergency Medicine, Stony Brook University Hospital
Coauthor(s): Elizabeth D Turner, MD, Clinical Assistant Instructor and Resident Physician, Department of Emergency Medicine, State University of New York Stony Brook University Hospital
Contributor Information and Disclosures

Updated: Jun 24, 2009

Differential Diagnoses

Acute generalized exanthematous pustulosis
Drug reaction with eosinophilia
Burns, Chemical
Erythema Multiforme
Burns, Ocular
Phototoxic dermatologic reactions
Burns, Thermal
Staphylococcal Scalded Skin Syndrome
Conjunctivitis
Stevens-Johnson Syndrome
Corneal Ulceration and Ulcerative Keratitis
Toxic Shock Syndrome
Dermatitis, Exfoliative

Other Problems to Be Considered

Paraneoplastic pemphigus

Workup

Laboratory Studies

  • No definitive or specific emergent laboratory tests are indicated for toxic epidermal necrolysis (TEN). Basic laboratory tests may be helpful in planning symptomatic or supportive therapy.
    • Diffuse skin involvement may cause significant fluid loss and electrolyte abnormalities. Renal failure can result from hypovolemic shock or sepsis.
    • Surveillance cultures of blood, skin, and urine should be obtained.

Imaging Studies

  • No specific imaging studies are indicated for toxic epidermal necrolysis (TEN).
  • Chest radiography should be performed in the setting of respiratory distress because tracheobronchial inflammation may predispose to diffuse interstitial pulmonary disease or pneumonia.

Other Tests

  • Toxic epidermal necrolysis (TEN) is diagnosed by histopathologic analysis. Skin biopsy, harvested at the earliest possible stage, is important in establishing an accurate diagnosis and directing specific therapeutic modalities. Therefore, early involvement of a dermatologist and dermatopathologist is recommended. Biopsy samples in TEN typically demonstrate invasion of T lymphocytes in a perivascular inflammatory pattern, although this is not specific to toxic epidermal necrolysis (TEN) or Stevens-Johnson syndrome (SJS). The invasion of affected tissues by inflammatory cells is surprisingly limited and may be a result of the Fas-FasL–mediated apoptotic pathways, which suppress inflammatory responses to cell death. Detachment of the epidermis occurs at the basement membrane.

Procedures

  • Wound care for patients with toxic epidermal necrolysis (TEN) should be provided by trained personnel, ideally at dedicated burn centers. No clear consensus exists regarding the need for extensive debridement of detached epidermal layers. 
  • Skin biopsy, as discussed, is very useful in differentiating toxic epidermal necrolysis from other dermatologic disorders.

More on Toxic Epidermal Necrolysis

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

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

  2. 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].

  3. Bachot N, Roujeau JC. Differential diagnosis of severe cutaneous drug eruptions. Am J Clin Dermatol. 2003;4(8):561-72. [Medline].

  4. Pereira FA, Mudgil AV, Rosmarin DM. Toxic epidermal necrolysis. J Am Acad Dermatol. Feb 2007;56(2):181-200. [Medline].

  5. Abe R, Shimizu T, Shibaki A, Nakamura H, Watanabe H, Shimizu H. Toxic epidermal necrolysis and Stevens-Johnson syndrome are induced by soluble Fas ligand. Am J Pathol. May 2003;162(5):1515-20. [Medline].

  6. Posadas SJ, Padial A, Torres MJ, Mayorga C, Leyva L, Sanchez E. Delayed reactions to drugs show levels of perforin, granzyme B, and Fas-L to be related to disease severity. J Allergy Clin Immunol. Jan 2002;109(1):155-61. [Medline].

  7. Nassif A, Moslehi H, Le Gouvello S, et al. Evaluation of the potential role of cytokines in toxic epidermal necrolysis. J Invest Dermatol. 2004;123:850-5.

  8. Chung WH, Hung SI, Yang JY, Su SC, Huang SP, Wei CY. Granulysin is a key mediator for disseminated keratinocyte death in Stevens-Johnson syndrome and toxic epidermal necrolysis. Nat Med. Dec 2008;14(12):1343-50. [Medline].

  9. Abood GJ, Nickoloff BJ, Gamelli RL. Treatment strategies in toxic epidermal necrolysis syndrome: where are we at?. J Burn Care Res. Jan-Feb 2008;29(1):269-76. [Medline].

  10. Bastuji-Garin S, Fouchard N, Bertocchi M, Roujeau JC, Revuz J, Wolkenstein P. SCORTEN: a severity-of-illness score for toxic epidermal necrolysis. J Invest Dermatol. Aug 2000;115(2):149-53. [Medline].

  11. Chung WH, Hung SI, Hong HS, et al. Medical genetics: a marker for Stevens-Johnson syndrome. Nature. Apr 1 2004;428(6982):486. [Medline].

  12. Roujeau JC, Kelly JP, Naldi L, Rzany B, Stern RS, Anderson T. 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].

  13. Endorf FW, Cancio LC, Gibran NS. Toxic epidermal necrolysis clinical guidelines. J Burn Care Res. Sep-Oct 2008;29(5):706-12. [Medline].

  14. Garcia-Doval I, LeCleach L, Bocquet H, Otero XL, Roujeau JC. Toxic epidermal necrolysis and Stevens-Johnson syndrome: does early withdrawal of causative drugs decrease the risk of death?. Arch Dermatol. Mar 2000;136(3):323-7. [Medline].

  15. Palmieri TL, Greenhalgh DG, Saffle JR, et al. A multicenter review of toxic epidermal necrolysis treated in U.S. burn centers at the end of the twentieth century. J Burn Care Rehabil. Mar-Apr 2002;23(2):87-96. [Medline].

  16. Magina S, Lisboa C, Leal V, Palmares J, Mesquita-Guimaraes J. Dermatological and ophthalmological sequels in toxic epidermal necrolysis. Dermatology. 2003;207(1):33-6. [Medline].

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Further Reading

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Keywords

toxic epidermal necrolysis, TEN, Lyell disease, Lyell's disease, mucocutaneous exfoliative disease, erythema multiforme, EM, bullous erythema multiforme, Stevens-Johnson syndrome, SJS, mucocutaneous reaction, widespread erythema, necrosis, bullous detachment of the epidermis, SJS-TEN, TEN with spots, TEN without spots, drug-induced skin disorder, drug eruption, staphylococcal scalded skin syndrome, autoimmune disease 

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Contributor Information and Disclosures

Author

Gregory P Garra, DO, Clinical Assistant Professor, Department of Emergency Medicine, Stony Brook University School of Medicine; Residency Program Director, Department of Emergency Medicine, Stony Brook University Hospital
Gregory P Garra, DO is a member of the following medical societies: American College of Emergency Physicians and Society for Academic Emergency Medicine
Disclosure: Nothing to disclose.

Coauthor(s)

Elizabeth D Turner, MD, Clinical Assistant Instructor and Resident Physician, Department of Emergency Medicine, State University of New York Stony Brook University Hospital
Elizabeth D Turner, MD is a member of the following medical societies: American College of Emergency Physicians, Emergency Medicine Residents Association, and Society for Academic Emergency Medicine
Disclosure: Nothing to disclose.

Medical Editor

Theodore J Gaeta, DO, MPH, FACEP, Clinical Associate Professor, Department of Emergency Medicine, Joan and Sanford Weill Medical College at Cornell University; Vice Chairman and Program Director of Emergency Medicine Residency Program, Department of Emergency Medicine, New York Methodist Hospital; Academic Chair, Adjunct Professor, Department of Emergency Medicine, St George's University School of Medicine
Theodore J Gaeta, DO, MPH, FACEP is a member of the following medical societies: Alliance for Clinical Education, American College of Emergency Physicians, Clerkship Directors in Emergency Medicine, Council of Emergency Medicine Residency Directors, New York Academy of Medicine, and Society for Academic Emergency Medicine
Disclosure: Nothing to disclose.

Pharmacy Editor

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

Managing Editor

Mark L Plaster, MD, JD, Executive Editor, Emergency Physicians Monthly
Mark L Plaster, MD, JD is a member of the following medical societies: American Academy of Emergency Medicine and American College of Emergency Physicians
Disclosure: M L Plaster Publishing Co LLC Ownership interest Management position

CME Editor

John D Halamka, MD, MS, Associate Professor of Medicine, Harvard Medical School, Beth Israel Deaconess Medical Center; Chief Information Officer, CareGroup Healthcare System and Harvard Medical School; Attending Physician, Division of Emergency Medicine, Beth Israel Deaconess Medical Center
John D Halamka, MD, MS is a member of the following medical societies: American College of Emergency Physicians, American Medical Informatics Association, Phi Beta Kappa, and Society for Academic Emergency Medicine
Disclosure: Nothing to disclose.

Chief Editor

Rick Kulkarni, MD, Assistant Professor of Surgery, Section of Emergency Medicine, Yale-New Haven Hospital
Rick Kulkarni, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Emergency Medicine, American College of Emergency Physicians, American Medical Association, American Medical Informatics Association, Phi Beta Kappa, and Society for Academic Emergency Medicine
Disclosure: WebMD Salary Employment

 
 
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