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Stevens Johnson Sendromu Tanısı Alan Bir Çocuk Olgu

Year 2018, Volume: 16 Issue: 1, 115 - 124, 01.04.2018

Abstract

Stevens-Johnson sendromu (SJS) nadir görülen fakat hayatı tehdit edebilen mukokutanöz tutulumun
olduğu T hücre aracılı bir aşırı duyarlılık reaksiyonudur. Olguların çoğu ilaçlarla tetiklenmektedir.
Enfeksiyonlar, aşılar, kimyasallar, sistemik ve malign hastalıklar da etiyolojide rol alabilir. Hastalık
genellikle ateş, halsizlik, başağrısı, miyalji, artralji ve üst solunum yolu enfeksiyonu gibi prodromal
semptomlarla başlar. Stevens-Johnson sendromu için özel bir tedavi yöntemi bulunmamaktadır.
Hastalık tanısının hızla konması, sebep olan ilacın erken dönemde kesilmesi ve destek tedavisi tedavinin
temelini oluşturmaktadır. Sistemik steroid, intravenöz immünglobulin (IVIG) ve siklosporin gibi ilaçlar
da tedavi amacıyla kullanılmaktadır.
Stevens-Johnson sendromu farkındalığını arttırmak, erken tanı ve destek tedavinin önemini vurgulamak
amacıyla SJS tanısı alan bir olgu sunulmuştur.

ABSTRACT
Stevens-Johnson syndrome (SJS) is a rare but life-threatening, mucocutaneous, T cell mediated
hypersensitivity reaction. Most of the cases are triggered by a drug. Infections, vaccines, chemicals,
systemic or malign diseases may have also roles in etiology. Disease generally starts with prodromal
symptoms including fever, malaise, headache, myalgia or upper respiratory tract infection. There is no
specific treatment modality for SJS.
Diagnosing the disease rapidly, discontinuation of the suspected drug immediately, and supportive
therapy are the main steps in treatment. Medications like systemic steroids, intravenous immunglobulin
(IVIG) and cyclosporin are also used in treatment.
Our aim in presenting a case of SJS is to increase awareness of this disease, to emphasize importance of
immediate diagnosis and supportive therapy.

References

  • 1. 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.
  • 2. Stevens AM, Johnson F: A new eruptive fever associated with stomatitis and opthalmia. Report of two cases in children. Am J Dis Child 1922; 24:526.
  • 3. Chan HL, Stern RS, Arndt KA, Langlois J, Jick SS, Jick H, et al. The incidence of erythema multiforme, Stevens-Johnson syndrome, and toxic epidermal necrolysis: A population-based study with particular reference to reactions caused by drugs among outpatients Arch Dermatol 1990; 126:43–7.
  • 4. Roujeau JC, Stern RS. Severe adverse cutaneous reactions to drugs. N Engl J Med 1994; 331:1272–85.
  • 5. 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.
  • 6. Nassif A, Bensussan A, Dorothee G, Mami-Chouaib F, Bachot N, Bagot M, 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.
  • 7. Alfirevic A, Jorgensen AL, Williamson PR, Chadwick DW, Park BK, Pirmohamed M. HLA-B locus in Caucasian patients with carbamazepine hypersensitivity. Pharmacogenomics 2006;7:813–8.
  • 8. Letko E, Papaliodis DN, Papaliodis GN, Daoud YJ, Ahmed AR, Foster CS. Stevens-Johnson syndrome and toxic epidermal necrolysis: a review of the literature. Ann Allergy Asthma Immunol 2005; 94:419-36.
  • 9. Wei CY, Chung WH, Huang HW, Chen YT, Hung SI. Direct interaction between HLA-B and carbamazepine activates T cells in patients with Stevens-Johnson syndrome. J Allergy Clin Immunol 2012; 129:1562-9.
  • 10. Lee HY, Chung WH. Toxic epidermal necrolysis: the year in review. Curr Opin Allergy Clin Immunol 2013;13:330-6.
  • 11. Bircher AJ. Symptoms and danger signs in acute drug hypersensitivity. Toxicology 2005;209:201-7.
  • 12. Fritsch PO, Sidoroff A. Drug-induced Stevens-Johnson syndrome/toxic epidermal necrolysis. Am J Clin Dermatol 2000;1:349-60.
  • 13. Turan H, Vatansever S, Özdemir Ö, Canıtez Y, Sarıcaoğlu H. Çocuklarda Stevens Johnson Sendromu ve Toksik Epidermal Nekroliz. J Curr Pediatr 2008;6:104-10.
  • 14. Levi N, Bastuji-Garin S, Mockenhaupt M, Roujeau JC, Flahault A, Kelly JP, et al. Medications as risk factors of Stevens-Johnson syndrome and toxic epidermal necrolysis in children: a pooled analysis. Pediatrics 2009; 123:e297-304.
  • 15. Bygum A, Gregersen JW, Buus SK. Acetaminophen-induced toxic epidermal necrolysis in a child. Pediatr Dermatol 2004;21:236–8.
  • 16. Biswal S, Swayam SS. Stevens-Johnson syndrome-toxic epidermal necrolysis overlap syndrome: case report. Int J Dermatol 2014;53(8):1042-44.
  • 17. de Prost N, Ingen-Housz-Oro S, Duong Ta, Valeyrie-Allanore L, Legrand P, Wolkenstein P et al. Bacteremia in Stevens-Johnson syndrome and toxic epidermal necrolysis: epidemiology, risk factors, and predictive value of skin cultures. Medicine (Baltimore) 2010; 89:28-36.
  • 18. Rzany B, Hering O, Mockenhaupt M, Schröder W, Goerttler E, Ring J, et al. Histopathological and epidemiological characteristics of patients with erythema exudativum multiforme major, Stevens-Johnson syndrome and toxic epidermal necrolysis. Br J Dermatol 1996; 135:6-11.
  • 19. 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.
  • 20. Rappersberger K, Foedinger D. Treatment of erythema multiforme, Stevens-Johnson syndrome, and toxic epidermal necrolysis. Dermatologic Therapy 2002;15:397-408.
  • 21. Metry DW, Jung P, Levy ML. Use of intravenous immunoglobulin in children with stevensjohnson syndrome and toxic epidermal necrolysis: seven cases and review of the literature. Pediatrics 2003;112:1430-6.
  • 22. Kirchhof MG, Miliszewski MA, Sikora S, Papp A, Dutz JP. Retrospective review of StevensJohnson syndrome/toxic epidermal necrolysis treatment comparing intravenous immunoglobulin with cyclosporine. J Am Acad Dermatol. 2014;71(5):941-7.
  • 23. Akan A, Erkoçoğlu M, Kaya A, Toyran M, Civelek E, Savaş Z et al. Stevens-Johnson sendromu ve toksik epidermal nekrolizisli çocuk hastalarda intravenöz immünglobulin tedavisi ve literatürün gözden geçirilmesi. J Asthma Allergy Immunol 2013;11:197-203
  • 24. Viard I, Wehrli P, Bullani R, Schneider P, Holler N, Salomon D, et al. Inhibition of toxic epidermal necrolysis by blockade of CD95 with human intravenous immunoglobulin. Science. 1998;282:490-3.
  • 25. Lam NS, Yang YH, Wang LC, Lin YT, Chiang BL. Clinical characteristics of childhood erythema multiforme, Stevens-Johnson syndrome and toxic epidermal necrolysis in Taiwanese children. J Microbiol Immunol Infect 2004;37:366-70.
  • 26. Uysal Soyer O, Durmaz E, Akçören Z, Demirgüneş E, Şekerel BE. Toksik epidermal nekrolizis ve Steven-Johnson sendromunda intravenöz immünglobulin kullanımı. Çocuk Sağlığı ve Hastalıkları Dergisi 2010;53:136-40.
  • 27. Egan CA, Grant WJ, Morris SE, Saffle JR, Zone JJ. Plasmapheresis as an adjunct treatment in toxic epidermal necrolysis. J Am Acad Dermatol. 1999;40:458-61.
  • 28. Chen J, Wang B, Zeng Y, Xu H. High-dose intravenous immunoglobulins in the treatment of Stevens-Johnson syndrome and toxic epidermal necrolysis in Chinese patients: a retrospective study of 82 cases. Eur J Dermatol. 2010 Nov-Dec;20(6):743-7.
  • 29. Lalosevic J, Nikolic M, Gajic-Veljic M, Skiljevic D, Medenica L. Stevens-Johnson syndrome and toxic epidermal necrolysis: a 20-year single-center experience. Int J Dermatol 2015; 54(8):978-84.
Year 2018, Volume: 16 Issue: 1, 115 - 124, 01.04.2018

Abstract

References

  • 1. 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.
  • 2. Stevens AM, Johnson F: A new eruptive fever associated with stomatitis and opthalmia. Report of two cases in children. Am J Dis Child 1922; 24:526.
  • 3. Chan HL, Stern RS, Arndt KA, Langlois J, Jick SS, Jick H, et al. The incidence of erythema multiforme, Stevens-Johnson syndrome, and toxic epidermal necrolysis: A population-based study with particular reference to reactions caused by drugs among outpatients Arch Dermatol 1990; 126:43–7.
  • 4. Roujeau JC, Stern RS. Severe adverse cutaneous reactions to drugs. N Engl J Med 1994; 331:1272–85.
  • 5. 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.
  • 6. Nassif A, Bensussan A, Dorothee G, Mami-Chouaib F, Bachot N, Bagot M, 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.
  • 7. Alfirevic A, Jorgensen AL, Williamson PR, Chadwick DW, Park BK, Pirmohamed M. HLA-B locus in Caucasian patients with carbamazepine hypersensitivity. Pharmacogenomics 2006;7:813–8.
  • 8. Letko E, Papaliodis DN, Papaliodis GN, Daoud YJ, Ahmed AR, Foster CS. Stevens-Johnson syndrome and toxic epidermal necrolysis: a review of the literature. Ann Allergy Asthma Immunol 2005; 94:419-36.
  • 9. Wei CY, Chung WH, Huang HW, Chen YT, Hung SI. Direct interaction between HLA-B and carbamazepine activates T cells in patients with Stevens-Johnson syndrome. J Allergy Clin Immunol 2012; 129:1562-9.
  • 10. Lee HY, Chung WH. Toxic epidermal necrolysis: the year in review. Curr Opin Allergy Clin Immunol 2013;13:330-6.
  • 11. Bircher AJ. Symptoms and danger signs in acute drug hypersensitivity. Toxicology 2005;209:201-7.
  • 12. Fritsch PO, Sidoroff A. Drug-induced Stevens-Johnson syndrome/toxic epidermal necrolysis. Am J Clin Dermatol 2000;1:349-60.
  • 13. Turan H, Vatansever S, Özdemir Ö, Canıtez Y, Sarıcaoğlu H. Çocuklarda Stevens Johnson Sendromu ve Toksik Epidermal Nekroliz. J Curr Pediatr 2008;6:104-10.
  • 14. Levi N, Bastuji-Garin S, Mockenhaupt M, Roujeau JC, Flahault A, Kelly JP, et al. Medications as risk factors of Stevens-Johnson syndrome and toxic epidermal necrolysis in children: a pooled analysis. Pediatrics 2009; 123:e297-304.
  • 15. Bygum A, Gregersen JW, Buus SK. Acetaminophen-induced toxic epidermal necrolysis in a child. Pediatr Dermatol 2004;21:236–8.
  • 16. Biswal S, Swayam SS. Stevens-Johnson syndrome-toxic epidermal necrolysis overlap syndrome: case report. Int J Dermatol 2014;53(8):1042-44.
  • 17. de Prost N, Ingen-Housz-Oro S, Duong Ta, Valeyrie-Allanore L, Legrand P, Wolkenstein P et al. Bacteremia in Stevens-Johnson syndrome and toxic epidermal necrolysis: epidemiology, risk factors, and predictive value of skin cultures. Medicine (Baltimore) 2010; 89:28-36.
  • 18. Rzany B, Hering O, Mockenhaupt M, Schröder W, Goerttler E, Ring J, et al. Histopathological and epidemiological characteristics of patients with erythema exudativum multiforme major, Stevens-Johnson syndrome and toxic epidermal necrolysis. Br J Dermatol 1996; 135:6-11.
  • 19. 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.
  • 20. Rappersberger K, Foedinger D. Treatment of erythema multiforme, Stevens-Johnson syndrome, and toxic epidermal necrolysis. Dermatologic Therapy 2002;15:397-408.
  • 21. Metry DW, Jung P, Levy ML. Use of intravenous immunoglobulin in children with stevensjohnson syndrome and toxic epidermal necrolysis: seven cases and review of the literature. Pediatrics 2003;112:1430-6.
  • 22. Kirchhof MG, Miliszewski MA, Sikora S, Papp A, Dutz JP. Retrospective review of StevensJohnson syndrome/toxic epidermal necrolysis treatment comparing intravenous immunoglobulin with cyclosporine. J Am Acad Dermatol. 2014;71(5):941-7.
  • 23. Akan A, Erkoçoğlu M, Kaya A, Toyran M, Civelek E, Savaş Z et al. Stevens-Johnson sendromu ve toksik epidermal nekrolizisli çocuk hastalarda intravenöz immünglobulin tedavisi ve literatürün gözden geçirilmesi. J Asthma Allergy Immunol 2013;11:197-203
  • 24. Viard I, Wehrli P, Bullani R, Schneider P, Holler N, Salomon D, et al. Inhibition of toxic epidermal necrolysis by blockade of CD95 with human intravenous immunoglobulin. Science. 1998;282:490-3.
  • 25. Lam NS, Yang YH, Wang LC, Lin YT, Chiang BL. Clinical characteristics of childhood erythema multiforme, Stevens-Johnson syndrome and toxic epidermal necrolysis in Taiwanese children. J Microbiol Immunol Infect 2004;37:366-70.
  • 26. Uysal Soyer O, Durmaz E, Akçören Z, Demirgüneş E, Şekerel BE. Toksik epidermal nekrolizis ve Steven-Johnson sendromunda intravenöz immünglobulin kullanımı. Çocuk Sağlığı ve Hastalıkları Dergisi 2010;53:136-40.
  • 27. Egan CA, Grant WJ, Morris SE, Saffle JR, Zone JJ. Plasmapheresis as an adjunct treatment in toxic epidermal necrolysis. J Am Acad Dermatol. 1999;40:458-61.
  • 28. Chen J, Wang B, Zeng Y, Xu H. High-dose intravenous immunoglobulins in the treatment of Stevens-Johnson syndrome and toxic epidermal necrolysis in Chinese patients: a retrospective study of 82 cases. Eur J Dermatol. 2010 Nov-Dec;20(6):743-7.
  • 29. Lalosevic J, Nikolic M, Gajic-Veljic M, Skiljevic D, Medenica L. Stevens-Johnson syndrome and toxic epidermal necrolysis: a 20-year single-center experience. Int J Dermatol 2015; 54(8):978-84.
There are 29 citations in total.

Details

Primary Language Turkish
Journal Section Olgu
Authors

Burcu Tahire Köksal

Özlem Yılmaz Özbek This is me

Esra Özmen This is me

Merih Tepeoğlu This is me

Publication Date April 1, 2018
Published in Issue Year 2018 Volume: 16 Issue: 1

Cite

APA Köksal, B. T., Yılmaz Özbek, Ö., Özmen, E., Tepeoğlu, M. (2018). Stevens Johnson Sendromu Tanısı Alan Bir Çocuk Olgu. Güncel Pediatri, 16(1), 115-124.
AMA Köksal BT, Yılmaz Özbek Ö, Özmen E, Tepeoğlu M. Stevens Johnson Sendromu Tanısı Alan Bir Çocuk Olgu. Güncel Pediatri. April 2018;16(1):115-124.
Chicago Köksal, Burcu Tahire, Özlem Yılmaz Özbek, Esra Özmen, and Merih Tepeoğlu. “Stevens Johnson Sendromu Tanısı Alan Bir Çocuk Olgu”. Güncel Pediatri 16, no. 1 (April 2018): 115-24.
EndNote Köksal BT, Yılmaz Özbek Ö, Özmen E, Tepeoğlu M (April 1, 2018) Stevens Johnson Sendromu Tanısı Alan Bir Çocuk Olgu. Güncel Pediatri 16 1 115–124.
IEEE B. T. Köksal, Ö. Yılmaz Özbek, E. Özmen, and M. Tepeoğlu, “Stevens Johnson Sendromu Tanısı Alan Bir Çocuk Olgu”, Güncel Pediatri, vol. 16, no. 1, pp. 115–124, 2018.
ISNAD Köksal, Burcu Tahire et al. “Stevens Johnson Sendromu Tanısı Alan Bir Çocuk Olgu”. Güncel Pediatri 16/1 (April 2018), 115-124.
JAMA Köksal BT, Yılmaz Özbek Ö, Özmen E, Tepeoğlu M. Stevens Johnson Sendromu Tanısı Alan Bir Çocuk Olgu. Güncel Pediatri. 2018;16:115–124.
MLA Köksal, Burcu Tahire et al. “Stevens Johnson Sendromu Tanısı Alan Bir Çocuk Olgu”. Güncel Pediatri, vol. 16, no. 1, 2018, pp. 115-24.
Vancouver Köksal BT, Yılmaz Özbek Ö, Özmen E, Tepeoğlu M. Stevens Johnson Sendromu Tanısı Alan Bir Çocuk Olgu. Güncel Pediatri. 2018;16(1):115-24.