Advertisement

Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis

  • Farhan Huq
  • Talib Omer
  • Solomon Behar
Chapter

Abstract

Stevens-Johnson syndrome (SJS)/toxic epidermal necrolysis (TEN) is a life-threatening hypersensitivity reaction which is usually triggered by medications and carries a high mortality. It is typically characterized by a nonspecific flu-like prodrome, followed by round macular or patchy dusky erythematous lesions of the skin that coalesce and develop into bullae, resembling painful thermal burns. Additionally most patients will have mucus membrane involvement. SJS and TEN are a continuum of the same disease entity, whereas SJS is the less severe form of the spectrum with <10% body surface involvement, and TEN with more than 30% is the most severe form, carrying a mortality of 25–34%.

Early discontinuation of the offending drug is essential. Given its similarity to thermal burns, the treatment strategy is predominantly supportive in nature and ideally should occur in a specialized burn unit where the focus should be aggressive fluid resuscitation, early recognition of possible skin infections, pain control, and nutritional support.

Keywords

Stevens-Johnson syndrome (SJS) Toxic epidermal necrolysis (TEN) Lyell syndrome Nikolsky sign Asboe-Hansen sign Bullae Mucocutaneous Burn Mucosal Macular exanthem SCORTEN score 

Bibliography

  1. 1.
    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.e1–16–quiz203–4.  https://doi.org/10.1016/j.jaad.2013.05.002.CrossRefGoogle Scholar
  2. 2.
    Mockenhaupt M, Viboud C, Dunant A, et al. Stevens-Johnson syndrome and toxic epidermal necrolysis: assessment of medication risks with emphasis on recently marketed drugs. The EuroSCAR-study. J Invest Dermatol. 2008;128(1):35–44.  https://doi.org/10.1038/sj.jid.5701033.CrossRefPubMedGoogle Scholar
  3. 3.
    Harris V, Jackson C, Cooper A. Review of toxic epidermal necrolysis. Int J Mol Sci. 2016;17(12):2135.  https://doi.org/10.3390/ijms17122135.CrossRefPubMedCentralGoogle Scholar
  4. 4.
    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.e1–13–quiz185–6.  https://doi.org/10.1016/j.jaad.2013.05.003.CrossRefGoogle Scholar
  5. 5.
    Husain Z, Reddy BY, Schwartz RA. DRESS syndrome: part I. Clinical perspectives. J Am Acad Dermatol. 2013;68(5):693.e1–14–quiz706–8.  https://doi.org/10.1016/j.jaad.2013.01.033.CrossRefGoogle Scholar
  6. 6.
    Hsu DY, Brieva J, Silverberg NB, Silverberg JI. Morbidity and mortality of Stevens-Johnson syndrome and toxic epidermal necrolysis in United States adults. J Invest Dermatol. 2016;136(7):1387–97.  https://doi.org/10.1016/j.jid.2016.03.023.CrossRefPubMedGoogle Scholar
  7. 7.
    Frey N, Jossi J, Bodmer M, et al. The epidemiology of Stevens-Johnson syndrome and toxic epidermal necrolysis in the UK. J Invest Dermatol. 2017;137(6):1240–7.  https://doi.org/10.1016/j.jid.2017.01.031.CrossRefPubMedGoogle Scholar
  8. 8.
    Mittmann N, Knowles SR, Koo M, Shear NH, Rachlis A, Rourke SB. Incidence of toxic epidermal necrolysis and Stevens-Johnson syndrome in an HIV cohort: an observational, retrospective case series study. Am J Clin Dermatol. 2012;13(1):49–54.  https://doi.org/10.2165/11593240-000000000-00000.CrossRefPubMedGoogle Scholar
  9. 9.
    Schneider JA, Cohen PR. Stevens-Johnson syndrome and toxic epidermal necrolysis: a concise review with a comprehensive summary of therapeutic interventions emphasizing supportive measures. Adv Ther. 2017;34(6):1235–44.  https://doi.org/10.1007/s12325-017-0530-y.CrossRefPubMedPubMedCentralGoogle Scholar
  10. 10.
    Harr T, French LE. Toxic epidermal necrolysis and Stevens-Johnson syndrome. Orphanet J Rare Dis. 2010;5(1):39.  https://doi.org/10.1186/1750-1172-5-39.CrossRefPubMedPubMedCentralGoogle Scholar
  11. 11.
    Downey A, Jackson C, Harun N, Cooper A. Toxic epidermal necrolysis: review of pathogenesis and management. J Am Acad Dermatol. 2012;66(6):995–1003.  https://doi.org/10.1016/j.jaad.2011.09.029.CrossRefPubMedGoogle Scholar
  12. 12.
    Baccaro LM, Sakharpe A, Miller A, Amani H. The first reported case of ureteral perforation in a patient with severe toxic epidermal necrolysis syndrome. J Burn Care & Res: Off Publ Am Burn Assoc. 2014;35(4):e265–8.  https://doi.org/10.1097/BCR.0b013e31829a4374.CrossRefGoogle Scholar
  13. 13.
    Saeed HN, Chodosh J. Ocular manifestations of Stevens-Johnson syndrome and their management. Curr Opin Ophthalmol. 2016;27(6):522–9.  https://doi.org/10.1097/ICU.0000000000000312.CrossRefPubMedGoogle Scholar
  14. 14.
    Jeanmonod P, Hubbuch M, Grünhage F, et al. Graft-versus-host disease or toxic epidermal necrolysis: diagnostic dilemma after liver transplantation. Transpl Infect Dis. 2012;14(4):422–6.  https://doi.org/10.1111/j.1399-3062.2012.00746.x.CrossRefPubMedGoogle Scholar
  15. 15.
    Yu J, Brandling-Bennett H, Co DO, Nocton JJ, Stevens AM, Chiu YE. Toxic epidermal necrolysis-like cutaneous lupus in pediatric patients: a case series and review. Pediatrics. 2016;137(6):e20154497-e20154497.  https://doi.org/10.1542/peds.2015-4497.CrossRefGoogle Scholar
  16. 16.
    Williams PM, Conklin RJ. Erythema multiforme: a review and contrast from Stevens-Johnson syndrome/toxic epidermal necrolysis. Dent Clin N Am. 2005;49(1):67–76–viii.  https://doi.org/10.1016/j.cden.2004.08.003.CrossRefGoogle Scholar
  17. 17.
    Lim VM, Do A, Berger TG, et al. A decade of burn unit experience with Stevens-Johnson syndrome/toxic epidermal necrolysis: clinical pathological diagnosis and risk factor awareness. Burns: J Int Soc Burn Injuries. 2016;42(4):836–43.  https://doi.org/10.1016/j.burns.2016.01.014.CrossRefGoogle Scholar
  18. 18.
    Pereira FA, Mudgil AV, Rosmarin DM. Toxic epidermal necrolysis. J Am Acad Dermatol. 2007;56(2):181–200.  https://doi.org/10.1016/j.jaad.2006.04.048.CrossRefPubMedGoogle Scholar
  19. 19.
    Naik H, Lockwood S, Saavedra A. A pilot study comparing histological and immunophenotypic patterns in stage 4 skin graft vs host disease from toxic epidermal necrolysis. J Cutan Pathol. 2017;1(7797):268–4.  https://doi.org/10.1111/cup.12986.CrossRefGoogle Scholar
  20. 20.
    Cices AD, Carneiro C, Majewski S, et al. Differentiating skin rash after stem cell transplantation: graft versus host disease, cutaneous reactions to drugs and viral exanthema. Curr Derm Rep. 2016;5(1):12–7.  https://doi.org/10.1007/s13671-016-0126-9.CrossRefGoogle Scholar
  21. 21.
    Correia O, Delgado L, Barbosa IL, et al. CD8+ lymphocytes in the blister fluid of severe acute cutaneous graft-versus-host disease: further similarities with toxic epidermal necrolysis. Dermatology (Basel). 2001;203(3):212–6.CrossRefGoogle Scholar
  22. 22.
    Wiadrowski TP, Reid CM. Drug-induced linear IgA bullous disease following antibiotics. Australas J Dermatol. 2001;42(3):196–9.CrossRefPubMedGoogle Scholar
  23. 23.
    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):149–53.  https://doi.org/10.1046/j.1523-1747.2000.00061.x.CrossRefPubMedGoogle Scholar
  24. 24.
    Guégan S, Bastuji-Garin S, Poszepczynska-Guigné E, Roujeau J-C, Revuz J. Performance of the SCORTEN during the first five days of hospitalization to predict the prognosis of epidermal necrolysis. J Invest Dermatol. 2006;126(2):272–6.  https://doi.org/10.1038/sj.jid.5700068.CrossRefPubMedGoogle Scholar
  25. 25.
    McCullough M, Burg M, Lin E, Peng D, Garner W. Steven Johnson syndrome and toxic epidermal necrolysis in a burn unit: a 15-year experience. Burns: J Int Soc Burn Injuries. 2017;43(1):200–5.  https://doi.org/10.1016/j.burns.2016.07.026.CrossRefGoogle Scholar
  26. 26.
    Atiyeh BS, Dham R, Yassin MF, El-Musa KA. Treatment of toxic epidermal necrolysis with moisture-retentive ointment: a case report and review of the literature. Dermatol Surg: Off Publ Am Soc Dermatol Surg [et al]. 2003;29(2):185–8.Google Scholar
  27. 27.
    Mahar PD, Wasiak J, Cleland H, et al. Secondary bacterial infection and empirical antibiotic use in toxic epidermal necrolysis patients. J Burn Care & Res: Off Publ Am Burn Assoc. 2014;35(6):518–24.  https://doi.org/10.1097/BCR.0000000000000062.CrossRefGoogle Scholar
  28. 28.
    Schulz JT, Sheridan RL, Ryan CM, MacKool B, Tompkins RG. A 10-year experience with toxic epidermal necrolysis. J Burn Care Rehabil. 2000;21(3):199–204.CrossRefPubMedGoogle Scholar
  29. 29.
    Gerull R, Nelle M, Schaible T. Toxic epidermal necrolysis and Stevens-Johnson syndrome: a review. Crit Care Med. 2011;39(6):1521–32.  https://doi.org/10.1097/CCM.0b013e31821201ed.CrossRefPubMedGoogle Scholar
  30. 30.
    Kardaun SH, Jonkman MF. Dexamethasone pulse therapy for Stevens-Johnson syndrome/toxic epidermal necrolysis. Acta Derm Venereol. 2007;87(2):144–8.  https://doi.org/10.2340/00015555-0214.CrossRefPubMedGoogle Scholar
  31. 31.
    Hirahara K, Kano Y, Sato Y, et al. Methylprednisolone pulse therapy for Stevens-Johnson syndrome/toxic epidermal necrolysis: clinical evaluation and analysis of biomarkers. J Am Acad Dermatol. 2013;69(3):496–8.  https://doi.org/10.1016/j.jaad.2013.04.007.CrossRefPubMedGoogle Scholar
  32. 32.
    Viard I, Wehrli P, Bullani R, et al. Inhibition of toxic epidermal necrolysis by blockade of CD95 with human intravenous immunoglobulin. Science. 1998;282(5388):490–3.CrossRefPubMedGoogle Scholar
  33. 33.
    Barron SJ, Del Vecchio MT, Aronoff SC. Intravenous immunoglobulin in the treatment of Stevens-Johnson syndrome and toxic epidermal necrolysis: a meta-analysis with meta-regression of observational studies. Int J Dermatol. 2015;54(1):108–15.  https://doi.org/10.1111/ijd.12423.CrossRefPubMedGoogle Scholar
  34. 34.
    Zimmermann S, Sekula P, Venhoff M, et al. Systemic Immunomodulating therapies for Stevens-Johnson syndrome and toxic epidermal necrolysis. JAMA Dermatol. 2017;153(6):514–9.  https://doi.org/10.1001/jamadermatol.2016.5668.CrossRefPubMedPubMedCentralGoogle Scholar
  35. 35.
    Enk AH, Hadaschik EN, Eming R, et al. European guidelines (S1) on the use of high-dose intravenous immunoglobulin in dermatology. J Eur Acad Dermatol Venereol. 2016;30(10):1657–69.  https://doi.org/10.1111/jdv.13725.CrossRefPubMedGoogle Scholar
  36. 36.
    Valeyrie-Allanore L, Wolkenstein P, Brochard L, et al. Open trial of ciclosporin treatment for Stevens-Johnson syndrome and toxic epidermal necrolysis. 2010;163(4):847–53.  https://doi.org/10.1111/j.1365-2133.2010.09863.x.
  37. 37.
    Hunger RE, Hunziker T, Buettiker U, Braathen LR, Yawalkar N. Rapid resolution of toxic epidermal necrolysis with anti-TNF-α treatment. J Allergy Clin Immunol. 2005;116(4):923–4.  https://doi.org/10.1016/j.jaci.2005.06.029.CrossRefPubMedGoogle Scholar
  38. 38.
    Scott-Lang V, Tidman M, McKay D. Toxic epidermal necrolysis in a child successfully treated with infliximab. Pediatr Dermatol. 2012;31(4):532–4.  https://doi.org/10.1111/pde.12029.CrossRefPubMedGoogle Scholar
  39. 39.
    Zárate-Correa LC, Carrillo-Gómez DC, Ramírez-Escobar AF, Serrano-Reyes C. Toxic epidermal necrolysis successfully treated with infliximab. J Investig Allergol Clin Immunol. 2013;23(1):61–3.PubMedGoogle Scholar
  40. 40.
    Fischer M, Fiedler E, Marsch WC, Wohlrab J. Antitumour necrosis factor-alpha antibodies (infliximab) in the treatment of a patient with toxic epidermal necrolysis. Br J Dermatol. 2002;146(4):707–9.  https://doi.org/10.1046/j.1365-2133.2002.46833.x.CrossRefPubMedGoogle Scholar
  41. 41.
    Paradisi A, Abeni D, Bergamo F, Ricci F, Didona D, Didona B. Etanercept therapy for toxic epidermal necrolysis. J Am Acad Dermatol. 2014;71(2):278–83.  https://doi.org/10.1016/j.jaad.2014.04.044.CrossRefPubMedGoogle Scholar
  42. 42.
    Wolkenstein P, Latarjet J, Roujeau J-C, et al. Randomised comparison of thalidomide versus placebo in toxic epidermal necrolysis. Lancet. 1998;352(9140):1586–9.  https://doi.org/10.1016/S0140-6736(98)02197-7.CrossRefPubMedGoogle Scholar
  43. 43.
    de Sica-Chapman A, Williams G, Soni N, Bunker CB. Granulocyte colony-stimulating factor in toxic epidermal necrolysis (TEN) and Chelsea & Westminster TEN management protocol [corrected]. Br J Dermatol. 2010;162(4):860–5.  https://doi.org/10.1111/j.1365-2133.2009.09585.x.CrossRefPubMedGoogle Scholar
  44. 44.
    Hsu DY, Brieva J, Silverberg NB, Paller AS, Silverberg JI. Pediatric Stevens-Johnson syndrome and toxic epidermal necrolysis in the United States. J Am Acad Dermatol. 2017;76(5):811–817.e814.  https://doi.org/10.1016/j.jaad.2016.12.024.CrossRefPubMedPubMedCentralGoogle Scholar
  45. 45.
    Hinc-Kasprzyk J, Polak-Krzemińska A, Głowacka M. Ożóg-Zabolska I. The use of plasmapheresis in a 4-year-old boy with toxic epidermal necrosis. Anaesthesiol Intensive Ther. 2015;47(3):210–3.  https://doi.org/10.5603/AIT.2015.0034.CrossRefPubMedGoogle Scholar
  46. 46.
    Wang Y-M, Tao Y-H, Feng T, Li H. Beneficial therapeutic effects of hemoperfusion in the treatment of severe Stevens-Johnson syndrome/toxic epidermal necrolysis: preliminary results. Eur Rev Med Pharmacol Sci. 2014;18(23):3696–701.PubMedGoogle Scholar
  47. 47.
    Yang C, Cho Y, Chen K, Chen Y, Song H, Chu C. Long-term sequelae of Stevens-Johnson syndrome/toxic epidermal necrolysis. Acta Derm Venereol. 2016;96(4):525–9.  https://doi.org/10.2340/00015555-2295.CrossRefPubMedGoogle Scholar
  48. 48.
    Saeed H, Mantagos IS, Chodosh J. Complications of Stevens-Johnson syndrome beyond the eye and skin. Burns : J Int Soc Burn Injuries. 2016;42(1):20–7.  https://doi.org/10.1016/j.burns.2015.03.012.CrossRefGoogle Scholar
  49. 49.
    Kohanim S, Palioura S, Saeed HN, et al. Acute and chronic ophthalmic involvement in Stevens-Johnson syndrome/toxic epidermal necrolysis - a comprehensive review and guide to therapy. II. Ophthalmic disease. Ocul Surf. 2016;14(2):168–88.  https://doi.org/10.1016/j.jtos.2016.02.001.CrossRefPubMedGoogle Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  • Farhan Huq
    • 1
  • Talib Omer
    • 2
  • Solomon Behar
    • 3
  1. 1.Department of DermatologyUniversity of MichiganAnn ArborUSA
  2. 2.Department of Emergency Medicine, Keck School of MedicineUniversity of Southern CaliforniaLos AngelesUSA
  3. 3.Pediatric Emergency MedicineLong Beach Memorial/Miller Children’s Hospital & Children’s Hospital Los AngelesLos AngelesUSA

Personalised recommendations