Advertisement

Stevens–Johnson Syndrome and Toxic Epidermal Necrolysis

  • Maja Mockenhaupt
  • Stevens–Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe, druginduced skin reactions with a high morbidity and mortality.

  • A consensus definition has allowed several epidemiological studies to estimate the risk of associated medications.

  • Drugs with a high risk to induce SJS and TEN are allopurinol, antibacterial sulfonamides including sulfasalazine, carbamazapine, lamotrigine, nevirapine, oxicam-NSAIDs, phenobarbital, and phenytoin.

  • Identification and withdrawal of the inducing drug(s) is crucial for the progression of the disease and the prognosis of the patient.

  • Supportive management is important to improve the patient's state, probably more than immunomodulating treatments such as intravenous immunoglobulin, corticosteroids etc. that have been tried so far.

Keywords

Intravenous Immunoglobulin Toxic Epidermal Necrolysis Bullous Pemphigoid Erythema Multiforme Johnson Syndrome 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. 1.
    Bastuji-Garin S, Rzany B, Stern RS et al (1993) Clinical classification of cases of toxic epidermal necrolysis, Stevens-Johnson syndrome, and erythema multiforme. Arch Dermatol 129:92–96PubMedCrossRefGoogle Scholar
  2. 2.
    Hebra von F (1866) Erythema exsudativum multiforme. In: Atlas der Hautkrankheiten, Kaiserliche Akademie der Wissenschaften. Wien, Austria, 54–57Google Scholar
  3. 3.
    Lyell A (1956) Toxic epidermal necrolysis: An eruption resembling scalding of the skin. Br J Dermatol 68:355–361PubMedCrossRefGoogle Scholar
  4. 4.
    Stevens AM, Johnson FC (1922) A new eruptive fever associated with stomatitis and ophthalmia: Report of two cases in children. Am J Dis Child 24:526–533Google Scholar
  5. 5.
    Assier H, Bastuji-Garin S, Revuz J et al (1995) Erythema multiforme with mucous membrane involvement and Stevens–Johnson syndrome are clinically different disorders with distinct causes. Arch Dermatol 131:539–543PubMedCrossRefGoogle Scholar
  6. 6.
    Auquier-Dunant A, Mockenhaupt M, Naldi L et al (2002) Correlations between clinical patterns and causes of erythema multiforme majus, Stevens-Johnson syndrome, and toxic epidermal necrolysis. Arch Dermatol 138:1019–1024PubMedCrossRefGoogle Scholar
  7. 7.
    Rzany B, Hering O, Mockenhaupt M et al (1996) Histopathological and epidemiological characteristics of patients with erythema exsudativum multiforme majus (EEMM), Stevens–Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN). Br J Dermatol 135:6–11PubMedCrossRefGoogle Scholar
  8. 8.
    Côté B, Wechsler J, Bastuji-Garin S et al (1995) Clinicopathologic correlation in erythema multiforme and Stevens–Johnson syndrome. Arch Dermatol 131:1268–1272PubMedCrossRefGoogle Scholar
  9. 9.
    Roujeau JC, Stern RS (1994) Severe adverse cutaneous reactions to drugs. New Eng J Med 331:1272–1285PubMedCrossRefGoogle Scholar
  10. 10.
    Sidoroff A, Dunant A, Viboud C et al (2007) Risk factors for acute generalized exanthematous pustulosis (AGEP) – results of a multinational case-control study (EuroSCAR). Br J Dermatol 157:989–996PubMedCrossRefGoogle Scholar
  11. 11.
    Ziemer M, Wiesend CL, Vetter R et al (2007) Cutaneous adverse drug reactions to valdecoxib distinct from Stevens-Johnson syndrome and toxic epidermal necrolysis. Arch Dermatol 143:711–716PubMedCrossRefGoogle Scholar
  12. 12.
    Elias PM, Fritsch P, Epstein EH Jr (1977) Staphylococcal scalded skin syndrome: Clinical features, pathogenesis, and recent microbiological and biochemical developments. Arch Dermatol 113:207–219PubMedCrossRefGoogle Scholar
  13. 13.
    Kauppinen K (1972) Cutaneous reactions to drugs with special reference to severe bullous mucocutaneous eruptions and sulphonamides. A clinical study. Acta Derm Venereol 52(Suppl.):68Google Scholar
  14. 14.
    Roujeau JC, Guillaume JC, Fabre JP et al (1990) Toxic epidermal necrolysis (Lyell's syndrome): Incidence and drug etiology in France, 1981–1985. Arch Dermatol 126:37–42PubMedCrossRefGoogle Scholar
  15. 15.
    Schöpf E, Stühmer A, Rzany B et al (1991) Toxic epidermal necrolysis and Stevens–Johnson syndrome. An epidemiologic study from West Germany. Arch Dermatol 127:839–842PubMedCrossRefGoogle Scholar
  16. 16.
    Rzany B, Mockenhaupt M, Baur S et al (1996) Epidemiology of erythema exsudativum multiforme majus (EEMM), Stevens–Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) in Germany (1990–1992). Structure and results of a population-based registry. J Clin Epidemiol 49:769–773PubMedCrossRefGoogle Scholar
  17. 17.
    Fagot JP, Mockenhaupt M, Bouwes Bavinck JN et al (2001) Nevirapine and the risk of Stevens–Johnson syndrome or toxic epidermal – results of a case-control study. AIDS 15:1–6CrossRefGoogle Scholar
  18. 18.
    Mockenhaupt M, Messenheimer J, Schlingmann J et al (2005) Risk of Stevens–Johnson syndrome and toxic epidermal necrolysis in new users of anti-epileptics. Neurology 64:1134–1138PubMedGoogle Scholar
  19. 19.
    Roujeau JC, Kelly JP, Naldi L et al (1995) Medication use and the risk of Stevens–Johnson syndrome or toxic epidermal necrolysis. N Eng J Med 333:1600–1607CrossRefGoogle Scholar
  20. 20.
    Mockenhaupt M, Viboud C, Dunant A et al (2008) Stevens– Johnson syndrome and toxic epidermal necrolysis: Assessment of medication risks with emphasis on recently marketed drugs. The EuroSCAR study. J Invest Dermatol 128(1):35–44PubMedCrossRefGoogle Scholar
  21. 21.
    Halevy S, Ghislain PD, Mockenhaupt M et al (2008) Allopurinol is the most common cause of Stevens–Johnson syndrome and toxic epidermal necrolysis in Europe and Israel. J Am Acad Dermatol 58(1):25–32PubMedCrossRefGoogle Scholar
  22. 22.
    Correia O, Delgado L, Ramos JP et al (1993) Cutaneous T-cell recruitment in toxic epidermal necrolysis: Further evidence of CD8 + lymphocyte involvement. Arch Dermatol 129:466–468PubMedCrossRefGoogle Scholar
  23. 23.
    Viard I, Wehrli P, Bullani R et al (1998) Inhibition of toxic epidermal necrolysis by blockade of CD95 with human intravenous immunoglobulin. Science 282:490–493PubMedCrossRefGoogle Scholar
  24. 24.
    Dietrich A, Kawakubo Y, Rzany B, et al (1995) Low N-acety-lating capacity in patients with Stevens–Johnson syndrome and toxic epidermal necrolysis. Exp Dermatol 4:313–316PubMedCrossRefGoogle Scholar
  25. 25.
    Nassif A, Bensussan A, Boumsell L et al (2004) Toxic epidermal necrolysis: Effector cells are drug-specific cytotoxic T cells. J Allergy Clin Immunol 114(5): 1209–1215PubMedCrossRefGoogle Scholar
  26. 26.
    Nassif A, Moslehi H, Le Gouvello S et al (2004) Evaluation of the potential role of cytokines in toxic epidermal necrolysis J Invest Dermatol. 123(5):850–855PubMedCrossRefGoogle Scholar
  27. 27.
    Roujeau JC, Huynh TN, Bracq C et al (1987) Genetic susceptibility to toxic epidermal necrolysis. Arch Dermatol 123:1171–1173PubMedCrossRefGoogle Scholar
  28. 28.
    Wolkenstein P, Charue D, Laurent P et al (1995) Metabolic predisposition to cutaneous adverse drug reactions: Role in toxic epidermal necrolysis caused by sulfonamides and anticonvulsants. Arch Dermatol 131:544–551PubMedCrossRefGoogle Scholar
  29. 29.
    Chung WH, Hung SI, Hong HS et al (2004) A marker for Stevens–Johnson syndrome. Nature 428:486PubMedCrossRefGoogle Scholar
  30. 30.
    Lonjou C, Borot N, Sekula P et al (2008) A European study of HLA-B in Stevens–Johnson syndrome and toxic epidermal necrolysis related to five high-risk drugs. Pharmacogenet Genom 18(2):99–107CrossRefGoogle Scholar
  31. 31.
    Lonjou C, Thomas L, Borot N, et al (2006) A marker for Stevens–Johnson syndrome and toxic epidermal necrolysis: Ethnicity matters. Pharmacogenom J 6:265–268Google Scholar
  32. 32.
    Roujeau JC, Chosidow O, Saiag P et al (1990) Toxic epidermal necrolysis (Lyell's syndrome). J Am Acad Dermatol 23:1039–1058PubMedCrossRefGoogle Scholar
  33. 33.
    Wolkenstein P, Latarje J, Roujeau J et al (1998) Randomised comparison of thalidomide versus placebo in toxic epidermal necrolysis. Lancet 352:1586–1589PubMedCrossRefGoogle Scholar
  34. 34.
    Prins C, Kerdel FA, Padilla S et al (2003) Treatment of toxic epidermal necrolysis with high-dose intravenous immunoglobulins. Multicenter retrospective analysis of 48 consecutive cases. Arch Dermatol 139:26–32PubMedCrossRefGoogle Scholar
  35. 35.
    Trent JT, Kirsner RS, Romanelli P et al (2003) Analysis of intravenous immunoglobulin for the treatment of toxic epidermal necrolysis using SCORTEN. The University of Miami experience. Arch Dermatol 139:39–43PubMedCrossRefGoogle Scholar
  36. 36.
    Faye O, Roujeau JC (2005) Treatment of epidermal necrolysis with high-dose intravenous immunoglobulins (IVIG). Clinical experience to date. Drugs 65(15):2085–2090PubMedCrossRefGoogle Scholar
  37. 37.
    Bastuji-Garin S, Fouchard N, Bertocchi M et al (2000) SCORTEN: A severity-of-illness score for toxic epidermal necrolysis. J Invest Dermatol 115:149–153PubMedCrossRefGoogle Scholar
  38. 38.
    Bachot N, Revuz J, Roujeau JC (2003) Intravenous immunoglobulin treatment for Stevens-Johnson syndrome and toxic epidermal necrolysis. A prospective noncomparative study showing no benefiton mortality or progression. Arch Dermatol 139:33–36PubMedCrossRefGoogle Scholar
  39. 39.
    Brown KM, Silver GM, Halerz M et al (2004) Toxic epidermal necrolysis: Does immunoglobulin make a difference? J Burn Care Rehabil 25:81–88PubMedCrossRefGoogle Scholar
  40. 40.
    Shortt R, Gomes M, Mittman N et al (2004) Intravenous immunoglobulin does not improve outcome in toxic epidermal necrolysis. J Burn Care Rehabil 25:246–255PubMedCrossRefGoogle Scholar
  41. 41.
    Schneck J, Fagot JP, Sekula P et al (2008) 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 58(1):33–40PubMedCrossRefGoogle Scholar
  42. 42.
    Garcia-Doval I, LeCleach L, Bocquet H et al (2000) Toxic epidermal necrolysis and Stevens–Johnson syndrome. Does early withdrawal of causative drugs decrease the risk of death? Arch Dermatol 136:323–327PubMedCrossRefGoogle Scholar
  43. 43.
    Stern RS (2000) Improving the outcome of patients with toxic epidermal necrolysis and Stevens-Johnson syndrome. Arch Dermatol 136:410PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 2009

Authors and Affiliations

  • Maja Mockenhaupt
    • 1
  1. 1.Dokumentationszentrum schwerer Hautreaktionen (DZH)Universitäts-Hautklinik FreiburgFreiburgGermany

Personalised recommendations