Steven-Johnson sendromu ve toksik epidermal nekroliz

Akman-Karakaş, Ayşe; DİCLE, Özlem; ALPSOY, Erkan

Steven-Johnson sendromu ve toksik epidermal nekroliz

Erkan ALPSOY, (Akdeniz Üniversitesi, Tıp Fakültesi, Deri ve Zührevi Hastalıklar Anabilim Dalı, Antalya, Türkiye)

Özlem DİCLE, (Akdeniz Üniversitesi, Tıp Fakültesi, Deri ve Zührevi Hastalıklar Anabilim Dalı, Antalya, Türkiye)

Ayşe Akman KARAKAŞ (Akdeniz Üniversitesi, Tıp Fakültesi, Deri ve Zührevi Hastalıklar Anabilim Dalı, Antalya, Türkiye)

Türkderm-Deri Hastalıkları ve Frengi Arşivi

21 0

Yıl: 2010 Cilt: 44 Sayı: 4 Sayfa Aralığı: 180 - 186 Metin Dili: Türkçe İndeks Tarihi: 29-07-2022

Steven-Johnson sendromu ve toksik epidermal nekroliz

Öz:

Steven-Johnson sendromu (SJS) ve toksik epidermal nekroliz (TEN), nadir görülen, ancak yüksek ölüm hızına sahip, genellikle ilaçlara karşı gelişen ve yaygın epidermal nekrozla giden, birbiri ile yakın ilişkili bir grup hastalığı tanımlamak için kullanılır. Yaygın epidermis hasarının nedeni keratinositlerin apotozisidir. İlaçlar ya da metabolitlerinin hapten görevi görerek keratinositlerin yüzeyine bağlanmasının ve onları antijenik hale getirmesinin olayı başlattığı düşünülmektedir. İlaca özgül CD8(+) sitotoksik T hücrelerinin Fas/FasL ve perforin/granzim B yolağı ile keratinosit apotozisini tetikleyerek hastalığı başlattığı düşünülür. Çok sayıda ilaç etken olarak bildirilmişse de, en sık olarak sulfonamid grubu antibiyotikler, antikonvülzan ilaçlar, beta laktam grubu antibiyotikler, allopurinol, steroid olmayan antiinflamatuvar ilaçlar, nevirapin ve tiasetazon etken olarak saptanır. Erken tanı ve şüpheli ilaç ya da ilaçların hemen kesilmesi tedavinin en önemli basamaklarından birisidir. Ayırıcı tanıya giren diğer tabloların hızla dışlanması gerekir. Son yıllarda yerel ve sistemik tedavide kullanılan çok çeşitli seçeneklere rağmen ideal bir destekleyici bakım halen en önemli ve etkili tedavi yaklaşımıdır. TEN için geliştirilen bir ölçek olan SCORTEN hastalığın şiddetini ve buna bağlı olarak da ölüm hızını tahmin etmede yaygın olarak kullanılmaktadır. SCORTEN değeri 3 (ölüm hızı; %35,3) ve üzeri olan hastaların hızlı bir şekilde yanık ünitesi veya yoğun bakım ünitesine transfer edilmesi son derece önemlidir. Bu yazıda SJS-TEN yelpazesi güncel literatür bilgileri ışığında klinik özellikleri, etyopatogenez ve tedavisindeki gelişmelerle birlikte gözden geçirilmektedir.

Anahtar Kelime:

Konular: Dermatoloji

Steven-Johnson syndrome (SJS) and toxic epidermal necrolysis

Öz:

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare, life-threatening conditions with a high mortality rate. SJS and TEN are used to denote a group of disorders closely related to each other, characterized by extensive epidermal necrolysis, and usually induced by drugs. Keratinocyte apoptosis is the main reason for widespread epidermal detachment. Drugs or their methobolites can act as a hapten after binding to the keratinocyte surface and initiate cytotoxic immunological attack. Drug- specific CD8+cytotoxic T cells mediate keratinocyte apoptosis by the Fas/FasL pathway and perforin/granzyme pathway. Although numerous drugs have been noted as responsible, sulfonamide class of antibiotics, anticonvulsants, beta-lactam antibiotics, allopurinol, nonsteroidal anti-inflammatory drugs, nevirapine and thiacetazone are the most frequently reported causative ones. Early diagnosis and withdrawal of suspected drug or drugs are one of the most important steps in the treatment. Other diseases resembling SJS/TEN should be excluded as soon as possible. Although various topical and systemic treatments have recently been used, ideal supportive care is still the most important and effective therapeutic approach. SCORTEN, a scoring system used to predict mortality in TEN, has been widely used in recent years. Transfer to a burn unit or intensive care unit is recommended for patients with a SCORTEN 3 (mortality rate; 35.3%) or over. In this paper, we aimed to review clinical findings, aetiopathogenesis and treatment of these syndromes in the light of current literature.

Anahtar Kelime:

Konular: Dermatoloji

Belge Türü: Makale Makale Türü: Derleme Erişim Türü: Erişime Açık

1. Roujeau JC: The spectrum of Stevens-Johnson syndrome and toxic epidermal necrolysis: a clinical classification. J Invest Dermatol 1994;102:28-30.
2. Assier H, Bastuji-Garin S, Revuz J, Roujeau JC: Erythema multiforme with mucous membrane involvement and Stevens-Johnson syndrome are clinically different disorders with distinct causes. Arch Dermatol 1995;131:539-43.
3. Bastuji-Garin S, Rzany B, Stern RS, Shear NH, Naldi L, Roujeau JC: Clinical classification of cases of toxic epidermal necrolysis, Stevens-Johnson syndrome, and erythema multiforme. Arch Dermatol. 1993;129:92-6.
4. Alpsoy E: Stevens Johnson sendromu ve toksik epidermal nekrolizis. Pediyatrik Dermatoloji. Ed. Tüzün Y, Kotoğyan A, Serdaroğlu S, Çokuğraş H, Tüzün B, Mat MC. ‹stanbul, Nobel Tıp Kitabevleri, 2005;389-95.
5. Roujeau JC, Chosidow O, Saiag P, Guillaume JC: Toxic epidermal necrolysis(Lyell syndrome). J Am Acad Dermatol1990;23:1039-58.
6. Borchers AT, Lee JL, Naguwa SM, et al: Stevens-Johnson syndrome and toxic epidermal necrolysis. Autoimmun Rev 2008;7:598-605.
7. Dicle O, Yılmaz E, Alpsoy E: Stevens-Johnson Sendromu ve Toksik Epidermal Nekroliz: Retrospektif Bir Değerlendirme. Türkderm 2009;43:15-20.
8. Schopf E, Stuhmer A, Rzany B, Victor N, Zentgraf R, Kapp JF: Toxic epidermal necrolysis and Stevens-Johnson syndrome. An epidemiologic study from West Germany. Arch Dermatol 1991;127:839-42.
9. Roujeau JC, Stern RS: Severe adverse cutaneous reactions to drugs. N Engl J Med 1994;331:1272-85.
10. Yazar ﬁ, Tosun F, Yılmaz E: Antralin uygulanmasına bağlı toksik epidermal nekrolizis. Deri Hastalıkları ve Frengi Arşivi 1989;23:197-200.
11. Demiral AN, Yerebakan O, Simsir V, Alpsoy E: Amifostine induced toxic epidermal necrolysis during radiotherapy: a case report. Jpn J Clin Oncol 2002;32:477-9.
12. Rzany B, Mockenhaupt M, Stocker U, Hamouda O, Schopf E: Incidence of Stevens-Johnson syndrome and toxic epidermal necrolysis in patients with the acquired immunodeficiency syndrome in Germany. Arch Dermatol 1993;129:1059.
13. Duncan KO, Tigelaar RE, Bolognia JL: Stevens-Johnson syndrome limited to multiple sites of radiation therapy in a patient receiving phenobarbital. J. Am. Acad. Dermatol 1999;40:493-6.
14. Leaute-Labreze C, Lamireau T, Chawki D, Maleville J, Taieb A: Diagnosis, classification, and management of erythema multiforme and Stevens-Johnson syndrome. Arch Dis Child 2000;83:347-52.
15. Koh MJA, Tay YK: Stevens-Johnson syndrome and toxic epidermal necrolysis in Asian children. J Am Acad Dermatol 2010;62:54-60
16. Mockenhaupt M: Severe drug-induced skin reactions: clinical pattern, diagnostics and therapy. J Dtsch Dermatol Ges. 2009;7:142-60.
17. Roujeau JC, Huynh TN, Bracq C, Guillaume JC, Revuz J, Touraine R: Genetic susceptibility to toxic epidermal necrolysis. Arch Dermatol 1987;123:1171-3.
18. Chung WH, Hung SI, Hong HS, et al: Medical genetics: a marker for Stevens-Johnson syndrome. Nature 2004; 428:86.
19. Kaniwa N, Saito Y, Aihara M, et al: HLA-B locus in Japanese patients with antiepileptics and allopurinol-related Stevens-Johnson syndrome and toxic epidermal necrolysis. Pharmacogenomics 2008; 9:1617-22.
20. Ferrell PB Jr, McLeod HL: Carbamazepine, HLA-B-1502 and risk of Stevens-Johnson syndrome and toxic epidermal necrolysis: US FDA recommendations. Pharmacogenomics 2008; 9:1543-6.
21. Roujeau JC: Drug-induced toxic epidermal necrolysis. II. Current aspects. Clin Dermatol 1993;11:493-500.
22. Shear NH, Spielberg SP, Grant DM, Tang BK, Kalow W: Differences in metabolism of sulfonamides predisposing to idiosyncratic toxicity. Ann Intern Med 1986;105:179-84.
23. Paul C, Wolkenstein P, Adle H, Wechsler J, Garchon HJ, Revuz J, et al: Apoptosis as a mechanism of keratinocyte death in toxic epidermal necrolysis. Br J Dermatol 1996;134:710-4.
24. Akman A, Alpsoy E: Eritema Multiforme, Steven-Johnson Sendromu ve Toksik Epidermal Nekroliz (Lyell Sendromu). Türkiye Klinikleri J Surg Med Sci 2006;2:6-15.
25. Pereira FA, Mudgil AV, Rosmarin DM: Toxic epidermal necrolysis. J Am Acad Dermatol 2007;56:181-200.
26. Lebargy F, Wolkenstein P, Gisselbrecht M, Lange F, Fleury-Feith J, Delclaux C, et al: Pulmonary complications in toxic epidermal necrolysis: a prospective clinical study. Intensive Care Med 1997;23:1237-44.
27. De Rojas MV, Dart JKG,Saw VPJ: The naturel history of Stevens-Johnson syndrome: patterns of chronic ocular disease and the role systemic immunosuppressive therapy. Br J Ophthalmol 2007;91:1048-53.
28. Guendry J, Roujeau JC, Binaghi M, Soubrane G, Muraine M: Risk factors ror the development of ocular complications of Stevens Johnson syndrome and toxic epidermal necrolysis. Arch Dermatol 2009;145:157-62.
29. Roujeau JC, Kelly JP, Naldi L, Rzany B, Stern RS, Anderson T, et al: Medication use and the risk of Stevens-Johnson syndrome or toxic epidermal necrolysis. N Engl J Med 1995;14;333:1600-7.
30. Hanakawa Y, Schechter NM, Lin C, Garza L, Li H, Yamaguchi T, et al: Molecular mechanisms of blister formation in bullous impetigo and staphylococcal scalded skin syndrome. J Clin Invest 2002;110:53-60.
31. Wolf R, Matz H, Marcos B, Orion E: Drug rash with eosinophilia and systemic symptoms vs toxic epidermal necrolysis: the dilemma of classification. Clin Dermatol 2005;23:311-4.
32. Roujeau JC: Clinical heterogeneity of drug hypersensitivity. Toxicology 2005;209:123-9.
33. Ghislain PD, Roujeau JC: Treatment of severe drug reactions: Stevens-Johnson syndrome, toxic epidermal necrolysis and hypersensitivity syndrome. Dermatol Online J 2002;8:5.
34. Bastuji-Garin S, Fouchard N, Berocchi M, et al: SCORTEN: a severity of illness score for toxic epidermal necrolysis. J Invest Dermatol 2000;115:149-53.
35. Westly ED, Wechsler HL: Toxic epidermal necrolysis: granulocytic leukopenia as a prognostic indicator. Arch Dermatol 1984;120:721-6.
36. Engelhardt SL, Schurr MJ, Helgerson RB: Toxic epidermal necrolysis: an analysis of referral patterns and steroid usage. J Burn Care Rehabil 1997;18:520-4.
37. McGee T, Munster A: Toxic epidermal necrolysis syndrome: mortality rate reduced with early referral to regional bum center. Plast Reconstr Surg 1998;102:1018-22.
38. Kelemen JJ III, Cioffi WG, McManus WF, Mason AD Jr, Pruitt BA Jr: Bum center care for patients with toxic epidermal necrolysis. J Am Coll Surg 1995;180:273-8.
39. Revuz J, Penso D, Roujeau J-C, et al: Toxic epidermal necrolysis: clinical findings and prognosis factors in 87 patients. Arch Dermatol 1987;123:1160-5.
40. Guegan S, Bastuji-Garin S, Poszepczynska-Guigne´ E, et al: Performance of the SCORTEN during the first five days of hospitalization to predict the prognosis of epidermal necrolysis. J Invest Dermatol 2006;126:272-6.
41. Garcia-Doval I, LeCleach L, Bocquet H, Otero XL, Roujeau JC. Toxic epidermal necrolysis and Stevens-Johnson syndrome: does early withdrawal of causative drugs decrease the risk of death? Arch Dermatol 2000;136:323-7.
42. Chave TA, Mortimer NJ, Sladden MJ, Hall AP, Hutchinson PE: Toxic epidermal necrolysis: current evidence, practical management and future directions. Br J Dermatol 2005;153:241-53.
43. Schneck J, Fagot JP, Sekula P, et al: Effects of treatment 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;58:33-40.
44. Kardaun SH, Jonkman MF: Dexamethasone pulse therapy for Stevens-Johnson syndrome/toxic epidermal necrolysis. Acta Derm Venereol 2007;87:144-8.
45. Yeung CK, Lam LK, Chan HH: The timing of intravenous immunoglobulin therapy in Stevens-Johnson syndrome and toxic epidermal necrolysis. Clin Exp Dermatol 2005;30:600-2.
46. Mittmann N, Chan B, Knowles S, et al: Intravenous immunoglobulin use in patients with toxic epidermal necrolysis and StevensJohnson syndrome. Am J Clin Dermatol 2006;7:359-68.
47. Chaidemenos GC, Chrysomallis F, Sombolos K, Mourellou O, Ioannides D, Papakonstantinou M: Plasmapheresis in toxic epidermal necrolysis. Int J Dermatol 1997;36:218-21.
48. Renfro L, Grant-Kels JM, Daman LA: Drug-induced toxic epidermal necrolysis treated with cyclosporin. Int J Dermatol 1989;28:441-4.
49. Heng MC, Allen SG: Efficacy of cyclophosphamide in toxic epidermal necrolysis. Clinical and pathophysiologic aspects. J Am Acad Dermatol 1991;25:778-86.
50. Hunger RE, Hunziker T, Buettiker U, Braathen LR, Yawalkar N: Rapid resolution of toxic epidermal necrolysis with antiTNF-α treatment. J Allergy Clin Immunol 2005;116:923-4.
51. Goulden V, Goodfield MJ: Recombinant granulocyte colony-stimulating factor in the management of toxic epidermal necrolysis. Br J Dermatol 1996;135:305-6.

APA	ALPSOY E, DİCLE Ö, Akman-Karakaş A (2010). Steven-Johnson sendromu ve toksik epidermal nekroliz. , 180 - 186.
Chicago	ALPSOY Erkan,DİCLE Özlem,Akman-Karakaş Ayşe Steven-Johnson sendromu ve toksik epidermal nekroliz. (2010): 180 - 186.
MLA	ALPSOY Erkan,DİCLE Özlem,Akman-Karakaş Ayşe Steven-Johnson sendromu ve toksik epidermal nekroliz. , 2010, ss.180 - 186.
AMA	ALPSOY E,DİCLE Ö,Akman-Karakaş A Steven-Johnson sendromu ve toksik epidermal nekroliz. . 2010; 180 - 186.
Vancouver	ALPSOY E,DİCLE Ö,Akman-Karakaş A Steven-Johnson sendromu ve toksik epidermal nekroliz. . 2010; 180 - 186.
IEEE	ALPSOY E,DİCLE Ö,Akman-Karakaş A "Steven-Johnson sendromu ve toksik epidermal nekroliz." , ss.180 - 186, 2010.
ISNAD	ALPSOY, Erkan vd. "Steven-Johnson sendromu ve toksik epidermal nekroliz". (2010), 180-186.

APA	ALPSOY E, DİCLE Ö, Akman-Karakaş A (2010). Steven-Johnson sendromu ve toksik epidermal nekroliz. Türkderm-Deri Hastalıkları ve Frengi Arşivi, 44(4), 180 - 186.
Chicago	ALPSOY Erkan,DİCLE Özlem,Akman-Karakaş Ayşe Steven-Johnson sendromu ve toksik epidermal nekroliz. Türkderm-Deri Hastalıkları ve Frengi Arşivi 44, no.4 (2010): 180 - 186.
MLA	ALPSOY Erkan,DİCLE Özlem,Akman-Karakaş Ayşe Steven-Johnson sendromu ve toksik epidermal nekroliz. Türkderm-Deri Hastalıkları ve Frengi Arşivi, vol.44, no.4, 2010, ss.180 - 186.
AMA	ALPSOY E,DİCLE Ö,Akman-Karakaş A Steven-Johnson sendromu ve toksik epidermal nekroliz. Türkderm-Deri Hastalıkları ve Frengi Arşivi. 2010; 44(4): 180 - 186.
Vancouver	ALPSOY E,DİCLE Ö,Akman-Karakaş A Steven-Johnson sendromu ve toksik epidermal nekroliz. Türkderm-Deri Hastalıkları ve Frengi Arşivi. 2010; 44(4): 180 - 186.
IEEE	ALPSOY E,DİCLE Ö,Akman-Karakaş A "Steven-Johnson sendromu ve toksik epidermal nekroliz." Türkderm-Deri Hastalıkları ve Frengi Arşivi, 44, ss.180 - 186, 2010.
ISNAD	ALPSOY, Erkan vd. "Steven-Johnson sendromu ve toksik epidermal nekroliz". Türkderm-Deri Hastalıkları ve Frengi Arşivi 44/4 (2010), 180-186.