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Toxic Epidermal Necrolysis: Treatment & Medication

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

Treatment

Prehospital Care

Prehospital care for patients with toxic epidermal necrolysis (TEN) is similar to that for patients with burns.

  • In severe toxic epidermal necrolysis, the barrier function of the skin is compromised. Thus, contamination and evaporation must be minimized. The patient should be treated similarly to one with extensive burns, that is, with the application of sterile coverings.
  • Fluid and pulmonary status must be carefully monitored.

Emergency Department Care

The two most important elements in the treatment of toxic epidermal necrolysis (TEN) is discontinuation of the offending drug and admission to a burn unit.13 Early recognition, prompt withdrawal of the causative agent, and referral to a burn center is key to successful outcome. Evidence suggests that early withdrawal of the offending agent and rapid admission to a burn unit is associated with a more favorable prognosis.14,15 Drugs with longer half-lives and those with circulating active metabolites may cause worse disease. Similarly, disease may be prolonged in patients with impaired renal or hepatic function (N -acetylation) as in the absence of metabolism, these circulating agents may continue to cause disease long after the last does of medication.

Emergency department care should be directed at minimizing fluid and electrolyte loss and preventing secondary infection. Aggressive fluid and electrolyte management, pain control, and meticulous skin care are important. Fluid resuscitation is somewhat different from typical burn management in that patients with toxic epidermal necrolysis have less severe microvascular injury and therefore less of an inflammatory response. Clinicians should direct fluid resuscitation based on the physiologic endpoint of urine output of 0.5-1 mL/kg/h.13 Patients with extensive skin involvement require reverse isolation and a sterile environment.

  • Areas of skin erosion should be covered with nonadherent protective dressings such as petroleum gauze.
  • Respiratory distress may result from mucosal sloughing and edema and may necessitate endotracheal intubation and ventilation.
  • Silver sulfadiazine should be avoided because it is a sulfonamide derivative and may precipitate toxic epidermal necrolysis (TEN). Silver compounds not utilizing sulfadiazine or other sulfa medications should be used because they assist in wound healing and prevent infection and bacterial growth.
  • Antibiotic prophylaxis is not indicated unless sepsis or staphylococcal scalded skin syndrome is strongly suspected.
  • No specific treatment modality has been proven effective, including plasmapheresis, corticosteroids, cyclophosphamide, cyclosporin, TNF-alpha inhibitors, and intravenous immune globulin.4 Multiple studies have been completed and multiple studies are ongoing for each of these modalities. The studies have concluded that either the risk of the medication outweighs the benefit or the data are inconclusive to support its utilization. Therefore, there is a significant need for randomized control studies to further evaluate potential treatment modalities in toxic epidermal necrolysis (TEN).
  • Immediately discontinue any potentially offending medications (if identified).

Consultations

In general, patients with toxic epidermal necrolysis (TEN) benefit from a team approach to diagnosis and management, including a dermatologist, dermatopathologist, a burn surgeon, and an intensivist.

  • Patients with suspected toxic epidermal necrolysis should be admitted to a burn unit as quickly as possible.
  • Dermatologists may assist with diagnosis, biopsy, and inpatient treatment.
  • Inpatient ophthalmology consultation is useful for assisting in the treatment of ocular manifestations and long-term sequelae.
  • ENT or urology consultation may also be helpful in patients with significant mucous membrane involvement.

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

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].

Further Reading

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 

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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