Hopp til innhold
NHI.no
Annonse
Informasjon, tilstand

Stevens-Johnson syndrom og toksisk epidermal nekrolyse

Stevens-Johnson syndrom og toksisk epidermal nekrolyse er sjeldne, akutte, livstruende hudsykdommer som viser seg ved utbredt løsning av hudoverflaten. Tilstandene er oftest forårsaket av et legemiddel.

Medikamentell behandling

Sist oppdatert:

19. mars 2019

Hva er Stevens-Johnson syndrom og toksisk epidermal nekrolyse?

Stevens-Johnson syndrom og toksisk epidermal nekrolyse er sjeldne, akutte, livstruende hudsykdommer som viser seg ved utbredt løsning av hudoverflaten (epidermis) fra underhuden, og kraftig utslett/betennelse i slimhinner, ledsaget av feber og varierende grad av sykdom også i indre organer. Tilstanden er oftest forårsaket av et legemiddel. Tidlig diagnose, stans av behandlingen med det utløsende legemiddel og relevant støttebehandling er avgjørende for om pasienten overlever.

Annonse

Stevens-Johnsons syndrom (SJS) og toksisk epidermal nekrolyse (TEN) er samme sykdom, men SJS har mindre omfattende hudskade. Ved SJS ser man løsning/avflakning av mindre enn ti prosent av hudoverflaten, mens ved toksisk epidermal nekrolyse (TEN) omfattes mer enn 30 prosent av huden.

Det oppstår cirka fire til syv tilfeller per en million mennesker per år. I Norge ble det i 2013 meldt tre tilfeller av den alvorligste formen (TEN).

Tilstanden kan opptre i alle aldersgrupper, men den ses oftest hos eldre personer, beinmargstransplanterte og pasienter med systemisk lupus erytematosus eller HIV-infeksjon.

Dette dokumentet er basert på det profesjonelle dokumentet Stevens-Johnson syndrom og toksisk epidermal nekrolyse . Referanselisten for dette dokumentet vises nedenfor

  1. Bygum A, Andersen KE. Toksisk epidermal nekrolyse. Ugeskr Læger 2006; 168: 2645-9. PubMed
  2. High WA. Stevens-Johnson syndrome and toxic epidermal necrolysis: Pathogenesis, clinical manifestations, and diagnosis. UpToDate, last updated Mar 12, 2019. UpToDate
  3. Cohen V. Toxic epidermal necrolysis. Medscape, last updated Dec 20, 2018. emedicine.medscape.com
  4. Wolkenstein P, Revuz J. Toxic epidermal necrolysis. Dermatol Clin 2000; 18: 485-95. PubMed
  5. Letko E, Papaliodis DN, Papaliodis GN et al. Stevens-Johnson syndrome and toxic epidermal necrolysis: a review of the literature. Ann Allergy Asthma Immunol 2005; 94: 419-36. PubMed
  6. Chung WH, Hung SI, Yang JY, et al. Granulysin is a key mediator for disseminated keratinocyte death in Stevens-Johnson syndrome and toxic epidermal necrolysis. Nat Med 2008; 14: 1343. pmid:19029983 PubMed
  7. Caproni M, Torchia D, Schincaglia E, et al. Expression of cytokines and chemokine receptors in the cutaneous lesions of erythema multiforme and Stevens-Johnson syndrome/toxic epidermal necrolysis. Br J Dermatol 2006; 155: 722. pmid:16965421 PubMed
  8. Cattelan AM, Trevenzoli M, Sasset L et al. Toxic epidermal necrolysis induced by nevirapine therapy: description of two cases and review of the literature J Infect 2001; 43: 246-54.
  9. 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; 128:35. PubMed
  10. Aa E, Erythema multiforme, Steven-Johnson syndrom og toksisk epidermal nekrolyse. RELIS 2014, Nytt om bivirkinger.
  11. de Prost N, Ingen-Housz-Oro S, Duong Ta, et al. Bacteremia in Stevens-Johnson syndrome and toxic epidermal necrolysis: epidemiology, risk factors, and predictive value of skin cultures. Medicine (Baltimore) 2010; 89:28. PubMed
  12. Morales ME, Purdue GF, Verity SM, et al. Ophthalmic Manifestations of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis and Relation to SCORTEN. Am J Ophthalmol 2010; 150:505. PubMed
  13. de Prost N, Mekontso-Dessap A, Valeyrie-Allanore L, et al. Acute respiratory failure in patients with toxic epidermal necrolysis: clinical features and factors associated with mechanical ventilation. Crit Care Med 2014; 42:118. PubMed
  14. Jha AK, Goenka MK. Colonic involvement in Stevens-Johnson syndrome: a rare entity. Dig Endosc 2012; 24:382. PubMed
  15. Garcia-Doval I, LeCleach L, Bocquet H et al. Toxic epidermal necrolysis and Stevens-Johnson syndrome. Does early withdrawal of causative drugs decrease the risk of death? Arch Dermatol 2000; 136: 323-7. PubMed
  16. Chave TA, Mortimer NJ, Sladden MJ et al. Toxic epidermal necrolysis: current evidence, practical management and future directions. Br J Dermatol 2005; 153: 241-53. PubMed
  17. Arevalo JM, Lorente JA, Gonzalez-Herrada C et al. Treatment of toxic epidermal necrolysis with cyclosporin A. J Trauma 2000; 48: 473-8. PubMed
  18. Sekula P, Dunant A, Mockenhaupt M, et al. Comprehensive survival analysis of a cohort of patients with Stevens-Johnson syndrome and toxic epidermal necrolysis. J Invest Dermatol 2013; 133:1197. PubMed
  19. Majumdar S, Mockenhaupt M, Roujeau JC, Townshend AP. Interventions for toxic epidermal necrolysis. Cochrane Database of Systematic Reviews 2002, Issue 4. Art. No.: CD001435. DOI: 10.1002/14651858.CD001435. DOI
  20. High WA, Nirken MH, Roujeau J-C. Stevens-Johnson syndrome and toxic epidermal necrolysis: Management, prognosis, and long-term sequelae. UpToDate, last updated Mar 03, 2015. UpToDate
  21. Huang YC, Li YC, Chen TJ. The efficacy of intravenous immunoglobulin for the treatment of toxic epidermal necrolysis: a systematic review and meta-analysis. Br J Dermatol 2012; 167: 424-32. PubMed
  22. 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; 23:61. PubMed
  23. Paradisi A, Abeni D, Bergamo F, et al. Etanercept therapy for toxic epidermal necrolysis. J Am Acad Dermatol 2014; 71:278. PubMed
  24. Bamichas G, Natse T, Christidou F, et al. Plasma exchange in patients with toxic epidermal necrolysis. Ther Apher 2002; 6:225. PubMed
  25. Prins C, Kerdel FA, Padilla RS et al. Treatment of toxic epidermal necrolysis with high-dose intravenous immunoglobulins: multicenter retrospective analysis of 48 consecutive cases. Arch Dermatol 2003; 139: 26-32. PubMed
  26. Al-Mutairi N, Arun J, Osama NE et al. Prospective, noncomparative open study from Kuwait of the role of intravenous immunoglobulin in the treatment of toxic epidermal necrolysis. Int J Dermatol 2004; 43: 847-51. PubMed
  27. Tan AW, Thong BY, Yip LW et al. High-dose intravenous immunoglobulins in the treatment of toxic epidermal necrolysis: an Asian series. J Dermatol 2005; 32: 1-6. PubMed
  28. Campione E, Marulli GC, Carrozzo AM et al. High-dose intravenous immunoglobulin for severe drug reactions: efficacy in toxic epidermal necrolysis. Acta Derm Venereol 2003; 83: 430-2. PubMed
  29. Trent JT, Kirsner RS, Romanelli P et al. Analysis of intravenous immunoglobulin for the treatment of toxic epidermal necrolysis using SCORTEN: The University of Miami Experience. Arch Dermatol 2003; 139: 39-43. PubMed
  30. Barron SJ, Del Vecchio MT, Aronoff SC, et al. 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 Jan;54(1):108-15. PubMed
  31. Bastuji-Garin S, Fouchard N, Bertocchi M et al. SCORTEN: A severity-of-illness score for toxic epidermal necrolysis. J Invest Dermatol 2000; 115: 149-53. PubMed
  32. Bachot N, Revuz J, Roujeau JC. Intravenous immunoglobulin treatment for Stevens-Johnson syndrome and toxic epidermal necrolysis. Arch Dermatol 2003; 139: 33-6. PubMed
  33. Brown KM, Silver GM, Halerz M et al. Toxic epidermal necrolysis: does immunoglobulin make a difference? J Burn Care Rehabil 2004; 25: 81-8. PubMed
  34. Shortt R, Gomez M, Mittman N et al. Intravenous immunoglobulin does not improve outcome in toxic epidermal necrolysis. J Burn Care Rehabil 2004; 25: 246-55. PubMed
  35. Sekula P, Dunant A, Mockenhaupt M, et al. Comprehensive survival analysis of a cohort of patients with Stevens-Johnson syndrome and toxic epidermal necrolysis. J Invest Dermatol 2013; 133: 1197-204. pmid:23389396 PubMed
Annonse
Annonse