eMedicine Specialties > Emergency Medicine > Infectious Diseases

Toxic Epidermal Necrolysis: Follow-up

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

Follow-up

Further Inpatient Care

  • The mainstay of treatment of patients with toxic epidermal necrolysis (TEN) is supportive care until the epithelium regenerates. Supportive measures include isolation, fluid and electrolyte balance, nutritional support, pain management, and protective dressings. Anesthetic mouthwash may alleviate the discomfort associated with oral erosions.
  • Meticulous wound care is necessary to prevent secondary infection. Debate continues in the literature regarding whether or not to debride the wounds associated with toxic epidermal necrolysis. To date, no conclusive evidence supports early, late, or no debridement of the wounds. Cutaneous lesions heal in approximately 2 weeks; mucosal membrane lesions take longer.
  • To prevent ocular and vaginal complications associated with toxic epidermal necrolysis, recommendations are to apply topical lubricants as well as to perform manual separation of the affected areas daily.

Transfer

  • Patients with suspected toxic epidermal necrolysis (TEN) should be transferred to a burn unit for expert wound management and comprehensive multidisciplinary care.

Deterrence/Prevention

  • Patients must be counseled regarding the likely causative medication or agent, and they must be advised to avoid these medications and those of the same or similar classes in the future. 

Complications

  • Numerous complications of toxic epidermal necrolysis (TEN) can arise as a result of the widespread cutaneous and mucosal membrane inflammation and necrosis.
    • Skin: Epithelial loss predisposes to septicemia (Pseudomonas aeruginosa, Staphylococcus aureus, gram-negative species, and Candida albicans)
    • Mucosal membranes: Ulceration of various mucosal membranes may result in pain, scarring, and stricture formation. Affected surfaces include oral, ocular, and urogenital mucosa.
    • Pulmonary: Inflammation of respiratory epithelium may result in bronchial hypersecretion, hypoxemia, interstitial infiltrates, pulmonary edema, bacterial pneumonia, or bronchiolitis obliterans. Pulmonary embolism and acute respiratory distress syndrome (ARDS) have also been reported.
    • Gastrointestinal: GI hemorrhage results from intestinal inflammation.
    • Renal: Hypovolemia may result from poor oral intake. Renal hypoperfusion, acute tubular necrosis, and renal insufficiency may develop subsequent to septic shock.

Prognosis

  • The overall prognosis of toxic epidermal necrolysis (TEN) is poor, with a mortality rate as high as 50%. Major sequelae are generally limited to the affected organ systems, that is, the skin and mucosal membranes. Mucosal lesions characteristically heal with scarring.
    • Cutaneous: Scarring may occur in areas of infection or over pressure points. Postinflammatory hyperpigmentation and nail growth abnormalities are frequent sequelae.
    • Ocular: Complications generally result from abnormal keratinization of the tarsal conjunctiva. A Sjögrenlike syndrome with decreased lacrimal secretion causes dry eye and predisposes to corneal abrasions and corneal scarring with neovascularization. In addition, patients have been reported to have palpebral synechiae, entropion, or symblepharon (adhesion of the eyelids), and blindness.16
    • Oral: Oral and lip lesions usually heal without complications, but strictures of the throat and esophagus have been reported.
    • Genitalia: Vulvovaginal synechiae and phimosis have been reported in the literature.

Patient Education

Miscellaneous

Medicolegal Pitfalls

  • Failure to refer suspected cases of toxic epidermal necrolysis (TEN) to a burn unit. 
  • Failure to withdraw potentially offending drugs or institution of unnecessary pharmacologic therapies that may further exacerbate toxic epidermal necrolysis.

Special Concerns

  • Geriatric patients have an unfavorable prognosis.
 


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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