Mehmet KILIÇ, Erdal TASKIN, Ömer GÜNBEY, Fatma Betül GÜNBEY

Stevens-Johnson Sendromu/Toksik Epidermal Nekroliz ve İlaç Ateşi Birlikteliği olan Bir Olgu Sunumu

Steven-Johnson sendromu (SJS) ve toksik epidermal nekroliz (TEN), nadir görülen, ancak yüksek ölüm hızına sahip, genellikle ilaclara karşı gelişen ve yaygın epidermal nekrozla giden, birbiri ile yakın ilişkili bir grup hastalığı tanımlamak için kullanılır. Yaygın epidermis hasarının nedeni keratinositlerin apotozisidir. Etiyolojisi genellikle ilaçlara bağlıdır. İlaclar ya da metabolitlerinin hapten görevi görerek keratinositlerin yüzeyine bağlanmasının ve onları antijenik hale getirmesinin olayı başlattığı düşünülmektedir. Bu makalede, SJS tanısı konulan ve izleminde deri lezyonlarının özelikleri ile SJS/TEN overlap tablosuna dönüşen, tedavi ile şikâyetleri düzelen, daha sonra ilaca bağlı ateş şikâyeti gelişen 16 yaşında bir erkek olgu sunulmuştur. Erken tanı ve şüpheli ilaç ya da ilacların hemen kesilmesi tedavinin en önemli basamaklarından birisidir. Ayırıcı tanıya giren diğer tabloların ekarte edilmesi gerekir. Son yıllarda yerel ve sistemik tedavide kullanılan çok çeşitli seçeneklere rağmen destekleyici bakım halen en önemli ve etkili tedavi yaklaşımıdır. Bu yazıda SJS/TEN ve ilaç ateşi güncel literatur bilgileri ışığında klinik özellikleri, etiyopatogenez ve tedavisindeki gelişmelerle birlikte gözden geçirilmektedir.

With Combination of Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis and Drug Fever: A Case Report

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare, life-threatening conditions with a high mortality rate. SJS and TEN 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. Its etiology usually depends on drugs. Drugs or their metabolites can act as a hapten after binding to the keratinocyte surface and initiate a cytotoxic immunological attack. In this article, a 16-year-old male patient diagnosed with SJS and turned into SJS/TEN overlap with the characteristics of skin lesions during the follow-up, whose complaints improved with treatment and later developed drug-related fever, is presented. Early diagnosis and withdrawal of suspected drugs are some of the most important steps in the treatment. SJS/TEN should be excluded from other diseases similar to SJS/TEN. Although various topical and systemic treatments have recently been used, supportive care is still the most important and effective therapeutic approach. This paper aimed to review clinical findings, with advances in etiopathogenesis and treatment of SJS/TEN and drug fever in light of current literature.

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1. Hazin R, Ibrahimi OA, Hazin Mİ, Kimyai-Asadi A. Stevens-Johnson syndrome: pathogenesis, diagnosis, and management. Ann Medicine 2008; 40: 129-38.
2. Roujeau JC, Guillaume JC, Fabre JP et al. Toxic epidermal necrolysis (Lyell syndrome). Incidence and drug etiology in France, 1981-1985. Arch Dermatol 1990; 126: 37-42.
3. Stevens AM, Johnson PC. A new eruptive fever assosiated with stomatitis and ophtalmica: report of two cases in children. Am J Dis Child 1922; 24: 526-33.
4. Auyeung J, Lee M. Successful Treatment of Stevens- Johnson syndrome with Cyclosporine and Corticosteroid. Can J Hosp Pharm 2018; 71: 272- 5.
5. 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.
6. Belver MT, Michavila A, Bobolea I, Feito M, Bellón T, Quirce S. Severe delayed skin reactions related to drugs in the paediatric age group: a review of the subject by way of three cases (Stevens- Johnson syndrome, toxic epidermal necrolysis and DRESS). Allergol Immunopathol (Madr) 2016; 44: 83-95.
7. Roujeau JC. The spectrum of Stevens-Johnson síndrome and toxic epidermal necrolysis: a clinical classification. J Invest Dermatol 1994; 102: 28-30.
8. Kangal H, Gülez P, Hekimoğlu Ü, Hizarcıoğlu M. Toxic epidermal necrolysis-Stevens Johnson overlap syndrome: A case report. İzmir Dr. Behçet Uz Çocuk Hast Dergisi 2012; 2: 32-5.
9. Ferrandiz-Pulido C, Garcia-Patos V. A review of causes of Stevens-Johnson syndrome and toxic epidermal necrolysis in children. Arch Dis Child 2013; 98: 998-1003.
10. Yang SC, Hu S, Zhang SZ et al. Corrigendum to "The Epidemiology of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis in China". J Immunol Res 2018; 2018: 4154507.
11. Hanson MA. Drug fever. Remember to consider it in diagnosis. Postgrad Med 1991; 89: 167-70.
12. Johnson DJ, Cunha BA. Drug Fever. Infect Dis Clin North Am 1996; 10: 85- 91.
13. Mackowiak PA, LeMaistre CF. Drug fever: A critical appraisal of conventional concepts. An analysis of 51 episodes in two Dallas hospitals and 97 episodes reported in the English literature. Ann Intern Med 1987; 106: 728-33.
14. Lipsky BA, Hirschmann JV. Drug fever. JAMA 1981; 245: 851-4.
15. Patel RA, Gallagher JC, Drug Fever. Pharmacotherapy 2010; 30: 57-69.
16. Demircioğlu F, Öztürk Y, Ünal N, Köse S, Kahraman Ö. Answer: Drug Fever. SSK Tepecik Hast Derg 2004; 14: 137-8.
17. Mackowiak PA. Drug fever: mechanisms, maxims and misconceptions. Am J Med Sci 1987; 294: 275-86.
18. Harr T, French L. Toxic epidermal necrolysis and Stevens-Johnson syndrome. Orphanet J Rare Dis 2010; 5: 1-11.
19. Ordoñez L, Salgueiro E, Jimeno FJ, Manso G. Spontaneous reporting of Stevens-Johnson syndrome and toxic epidermal necrolysis associated with antiepileptic drugs. Eur Rev Med Pharmacol Sci 2015; 19: 2732-7.
20. Çekiç Ş, Canıtez Y, Sapan N. Evaluation of the patients diagnosed with Stevens Johnson syndrome and toxic epidermal necrolysis: a single center experience. Turk Pediatri Ars 2016; 51: 152-8.
21. Kılıç M, Taşkın E, Bulut M, Sarı Y, Aktaş G. A Case of Stevens-Johnson Syndrome Triggered by Combined Use of Lamotrigine and Valproic Acid. Fırat Tıp Dergisi 2010; 15: 101-3.
22. 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.
23. Schneck J, Fagot JP, Sekula P et al. 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 2008; 58: 33-40.
24. Prins C, Kerdel AF, Padilla RS, et al. TEN IVIG Study Group. Treatment of toxic epidermal necrolysis with high-dose intravenous immunoglobulins: multicenter retrospective analysis of 48 consecutive cases. Arch Dermatol 2003; 139: 26-32.