Core Dermatology Diagnoses / CategoriesInflammatory

Toxic epidermal necrolysis

Author: Deputy Editor:

Publish date: Posted on
Last updated: October 24, 2023

Keywords #

Toxic epidermal necrolysis
Steven-Johnson syndrome
Drug eruption

Diagnosis #

Toxic epidermal necrolysis (TEN) is a rare, life-threatening mucocutaneous disorder. TEN and Steven-Johnson syndrome (SJS) are variants of the same spectrum, distinguished by the percent of body surface area (BSA) involved, with TEN involving more than 30% of BSA.  TEN is T-cell mediated immune reaction that involves sloughing of the skin at the dermal-epidermal junction. The etiology is usually drug-induced, with the most common offending agents being antibacterials (sulfonamides), anticonvulsants, and allopurinol.1

Key Concepts #
  • TEN is a potentially fatal condition
  • Antibacterial, anticonvulsants, and allopurinol are the most common inciting agents
  • It is characterized by violaceous macules that develop into painful bullae, epidermal sloughing and skin necrosis
  • The mainstay of treatment is supportive care and stopping the offending agent
Epidemiology #

The incidence of TEN is about 0.4-1.9 per million. TEN affects patients of all age groups and is more frequently found in women and elderly.2,3,4   HIV patients have an increased risk to developing TEN, which may be explained by the increased exposure to drugs.2,5 Patients with a history of systemic lupus erythematosus and bone marrow transplantation may also be at higher risk.5  The mortality rate of TEN is 25% to 30% and the severity of the disease can be estimated using the Severity of Illness Score for Toxic Epidermal Necrolysis (SCORTEN) model.6  Sepsis from Staphylococcal aureus and Pseudomonas aeruginosa is the most common cause of death in TEN patients.5

Clinical Features #

TEN is characterized by widespread sloughing of skin and mucosal surfaces. The skin manifestations are typically preceded by a prodrome of fever, cough, anorexia, and pharyngitis. 1,2  Clinical features include erythematous, dusky, or violaceous macules and morbilliform or atypical targetoid lesions. Over a period of 24 hours to 2 weeks, the lesions evolve to painful bullae, epidermal sloughing, and skin necrosis with gray hue.2 TEN is also associated with a positive Nikolsky sign, which is the separation of the epidermis from the dermis with lateral pressure, and a positive Asboe-Hansen sign, the lateral extension of bullae with pressure.  Mucosal surfaces are involved in 87%-100% of TEN cases.2 TEN affects multiple organ systems as erosion and necrosis can occur in the conjunctivae, trachea, kidney, and gastrointestinal tract.

Differential Diagnoses #
  • Erythema multiforme
  • Staphylococcal scalded skin syndrome
  • Bullous pemphigoid
  • Burns
  • Acute generalized erythematous pustulosis
  • Generalized fixed bullous drug eruption
  • Drug reaction with eosinophilia and systemic symptoms
  • Bullous pemphigoid
  • Edematous erythroderma
  • Linear IgA bullous dermatosis
  • Lupus erythematosus
  • Paraneoplastic pemphigus
  • Exfoliative dermatitis

    • Exfoliative and blistering diseases are in the differential diagnosis of TEN. However, the etiology, patient history, clinical features, and skin biopsy will help distinguish TEN from other potential diagnoses.5

Diagnostic Workup #

The diagnosis of TEN is made based on both clinical and histopathologic findings. A biopsy of TEN lesions reveal full-thickness epidermal necrosis and a subepidermal split with lymphocytic infiltrate at the dermoepidermal junction.5

Treatment #

: Treatment of TEN involves withdrawal of the offending agent and transferring to a specialty unit or burn center to provide supportive care.  Supportive treatment includes wound care (silver dressings, biosynthetic dressings, or even with grafts), adequate enteral nutrition and intravenous fluid resuscitation, and ophthalmologic and Ob/Gyn consultation with ocular and genital tract involvement respectively.

There is currently no standardized management for systemic treatment of TEN. The use of corticosteroids and intravenous immunoglobulin (IVIG) have been inconclusive in the treatment of TEN.7–10  Immunosuppressive agents such as etanercept and cyclosporine are currently being studying. 9,11

Slide Viewer #
https://utahderm.med.utah.edu/image-viewer/?diagnosis=toxic_epidermal_necrolysis
References #
  1. Kelemen JJ, Cioffi WG, McManus WF, Mason AD, Pruitt BA. Burn center care for patients with toxic epidermal necrolysis. J Am Coll Surg. 1995;180(3):273-278.
  2. Schwartz RA, McDonough PH, Lee BW. Toxic epidermal necrolysis: Part I. Introduction, history, classification, clinical features, systemic manifestations, etiology, and immunopathogenesis. J Am Acad Dermatol. 2013;69(2):173.e1-173.e13. doi:10.1016/J.JAAD.2013.05.003
  3. Lissia M, Mulas P, Bulla A, Rubino C. Toxic epidermal necrolysis (Lyell’s disease). Burns. 2010;36(2):152-163. doi:10.1016/j.burns.2009.06.213
  4. Rzany B, Correia O, Kelly JP, Naldi L, Auquier A, Stern R. Risk of Stevens-Johnson syndrome and toxic epidermal necrolysis during first weeks of antiepileptic therapy: A case-control study. Lancet. 1999;353(9171):2190-2194. doi:10.1016/S0140-6736(98)05418-X
  5. Bachot N, Roujeau JC. Differential diagnosis of severe cutaneous drug eruptions. Am J Clin Dermatol. 2003;4(8):561-572. doi:10.2165/00128071-200304080-00006
  6. 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. 2000;115(2):149-153. doi:10.1046/j.1523-1747.2000.00061.x
  7. Pehr K. The EuroSCAR study: Cannot agree with the conclusions. J Am Acad Dermatol. 2008;59(5):898-899. doi:10.1016/j.jaad.2008.07.004
  8. Schneck J, Fagot JP, Sekula P, Sassolas B, Roujeau JC, Mockenhaupt M. Effects of treatments on the mortality of Stevens-Johnson syndrome and toxic epidermal necrolysis: A retrospective study on patients included in the prospective EuroSCAR Study. J Am Acad Dermatol. 2008;58(1):33-40. doi:10.1016/j.jaad.2007.08.039
  9. Schwartz RA, McDonough PH, Lee BW. Toxic epidermal necrolysis: Part II. Prognosis, sequelae, diagnosis, differential diagnosis, prevention, and treatment. J Am Acad Dermatol. 2013;69(2):187.e1-187.e16. doi:10.1016/J.JAAD.2013.05.002
  10. Endorf FW, Cancio LC, Gibran NS. Toxic epidermal necrolysis clinical guidelines. J Burn Care Res. 2008;29(5):706-712. doi:10.1097/BCR.0b013e3181848bb1
  11. White KD, Abe R, Ardern-Jones M, et al. SJS/TEN 2017: Building Multidisciplinary Networks to Drive Science and Translation. J Allergy Clin Immunol Pract. 2018;6(1):38-69. doi:10.1016/j.jaip.2017.11.023
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