Résumés
Résumé
Les syndromes de Stevens-Johnson et de Lyell sont regroupés sous le nom de nécrolyse épidermique. Il s’agit de maladies bulleuses mucocutanées aiguës, rares mais extrêmement graves, provoquées le plus souvent par une « allergie » médicamenteuse. Le mécanisme conduisant à la mort par apoptose brutale et disséminée des cellules de l’épithélium de la peau et des muqueuses a longtemps été un mystère. Les connaissances accumulées suggèrent toutefois l’existence d’un phénomène de cytotoxicité lymphocytaire dirigé contre des cellules épidermiques, reconnues comme étrangères après fixation du médicament inducteur de la réaction sur certaines molécules HLA de classe I. Tout semble se passer comme s’il s’agissait d’un rejet aigu d’une greffe d’épiderme.
Summary
Toxic epidermal necrolysis and Stevens-Johnson syndrome are acute and severe adverse reaction to drugs, characterized by the widespread destruction of the epithelium of the skin and mucous membranes. This destruction by massive apoptosis leads to a clinical pattern of epidermal necrolysis resembling second degree burns, with sheets of necrotic epidermis detached from the underlying dermis. The mechanisms of acute and extensive destruction of the skin are not yet fully understood. At the onset of the reaction blisters develop from the fluid that accumulates between the dead epidermis and the dermis. High concentrations of mononuclear cells are present in this blister fluid, principally CD8 T-lymphocytes that may exhibit a drug specific MHC class I restricted cytotoxicity against autologous cells. The intervention of soluble mediators such as TNFα, perforin/granzyme, or Fas-Ligand may be necessary for amplifying the apoptosis of keratinocytes. A strong association between epidermal necrolysis to certain drugs and rare HLA-B genotypes suggests that direct interaction between these drugs and HLA-B molecules may initiate a reaction resembling the acute rejection of allogeneic epidermis.
Parties annexes
Références
- 1. Bastuji-Garin S, Rzany B, Stern RS, et al. Clinical classification of cases of toxic epidermal necrolysis, Stevens-Johnson syndrome, and erythema multiforme. Arch Dermatol 1993 ; 129 : 92-6.
- 2. Roujeau JC, Kelly JP, Naldi L, et al. Medication use and the risk of Stevens-Johnson syndrome or toxic epidermal necrolysis. N Engl J Med 1995 ; 333 : 1600-7.
- 3. Lyell A. Toxic epidermal necrolysis : an eruption resembling scalding of the skin. Br J Dermatol 1956 ; 68 : 355-61.
- 4. Merot Y, Saurat JH. Clues to pathogenesis of toxic epidermal necrolysis. Int J Dermatol 1985 ; 24 : 165-8.
- 5. Miyauchi H, Hosokawa H, Akaeda T, et al. T-cell subsets in drug-induced toxic epidermal necrolysis. Possible pathogenic mechanism induced by CD8-positive T cells. Arch Dermatol 1991 ; 127 : 851-5.
- 6. Villada G, Roujeau JC, Clerici T, et al. Immunopathology of toxic epidermal necrolysis. Keratinocytes, HLA-DR expression, Langerhans cells, and mononuclear cells : an immunopathologic study of five cases. Arch Dermatol 1992 ; 128 : 50-3
- 7. Correia O, Delgado L, Ramos JP, et al. Cutaneous T-cell recruitment in toxic epidermal necrolysis, further evidence of CD8+ lymphocyte involvement. Arch Dermatol1993 ; 129 : 466-8.
- 8. Paul C, Wolkenstein P, Adle H, et al. Apoptosis as a mechanism of keratinocyte death in toxic epidermal necrolysis. Br J Dermatol 1996 ; 134 : 710-4.
- 9. Paquet P, Nikkels A, Arrese JE, et al. Macrophages and tumor necrosis factor alpha in toxic epidermal necrolysis. Arch Dermatol 1994 ; 130 : 605-8.
- 10. Viard I, Wehrli P, Bullani R, et al. Inhibition of toxic epidermal necrolysis by blockade of CD95 with human intravenous immunoglobulin. Science 1998 ; 282 : 490-3.
- 11. Abe R, Shimizu T, Shibaki A, et al. Toxic epidermal necrolysis and Stevens-Johnson syndrome are induced by soluble Fas Ligand. Am J Pathol 2003 ; 162 : 1515-20.
- 12. Paquet P, Pierard GE. Soluble fractions of tumor necrosis factor alpha, interleukin-6 and their receptors in toxic epidermal necrolysis : a comparison with second-degree burns. Int J MolMed 1998 ; 1 : 459-62.
- 13. Wolkenstein P, Latarjet J, Roujeau JC, et al. Randomised comparison of thalidomide versus placebo in toxic epidermal necrolysis. Lancet 1998 ; 352 : 1586-9.
- 14. Rhodes LE, Hashim IA, McLaughlin PJ, Friedmann PS. Blister fluid cytokines in cutaneous inflammatory bullous disorders. Acta Dermatol Venereol 1999 ; 79 : 288-90.
- 15. Trent JT, Kirsner RS, Romanelli P, Kerdel FA. Use of SCORTEN to accurately predict mortality in patients with toxic epidermal necrolysis in the United States. Arch Dermatol2004 ; 140 : 890-2.
- 16. Bachot N, Revuz J, Roujeau JC. Intravenous immunoglobulin treatment for Stevens-Johnson syndrome and toxic epidermal necrolysis. Arch Dermatol 2003 ; 139 : 33-6.
- 17. Brown KM, Silver GM, Halerz M, et al. Toxic epidermal necrolysis : does immunoglobulin make a difference ? J Burn Care Rehabil 2004 ; 25 : 81-8.
- 18. 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.
- 19. Berthou C, Michel L, Soulie A, et al. Acquisition of granzyme B and Fas ligand proteins by human keratinocytes contributes to epidermal cell defense. J Immunol 1997 ; 159 : 5293-300.
- 20. Paquet P, Pierard GE. Keratinocyte injury in toxic epidermal necrolysis : simultaneous but distinct topographic expression of CD95R and calprotectin. Int J Mol Med 2002 ; 10 : 145-7.
- 21. Gerber BO, Pichler WJ. Cellular mechanisms of T cell mediated drug hypersensitivity. Curr Opin Immunol 2004 ; 16 : 732-7.
- 22. Le Cleach L, Delaire S, Boumsell L, et al. Blister fluid T lymphocytes during toxic epidermal necrolysis are functional cytotoxic cells which express human natural killer (NK) inhibitory receptors. Clin Exp Immunol 2000 ; 119 : 225-30.
- 23. Nassif A, Bensussan A, Dorothee G, et al. Drug specific cytotoxic T-cells in the skin lesions of a patient with toxic epidermal necrolysis. J Invest Dermatol 2002 ; 118 : 728-33.
- 24. Nassif A, Bensussan A, Boumsell L, et al. Toxic epidermal necrolysis : effector cells are drug-specific cytotoxic T cells. J Allergy Clin Immunol 2004 ; 114 : 1209-15.
- 25. 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.
- 26. Shresta S, Pham CTN, Thomas DA, et al. How do cytotoxic lymphocytes kill their targets ? Curr Opin Immunol 1998 ; 10 : 581-7.
- 27. Azukizawa H, Kosaka H, Sano S, et al. Induction of T-cell-mediated skin disease specific for antigen transgenically expressed in keratinocytes. Eur J Immunol 2003 ; 33 : 1879-88.
- 28. Chung WH, Hung SI, Hong HS, et al. A marker for Stevens-Johnson syndrome. Nature 2004 ; 428 : 86.
- 29. Hung SL, Chung WH, Liou LB, et al. HLA-B*5801 allele as a genetic marker for severe cutaneous adverse reactions caused by allopurinol. Proc Natl Acad Sci USA 2005 ; 102 : 4134-9.