Çocuklarda Steven’s Johnson Sendromu ve Toksik Epidermal Nekroliz

Öz

Amaç: Steven’s Johnson Sendromu SJS ve Toksik Epidermal Nekroliz TEN tanısı almış çocuk hastalarda klinik bulgular, sorumlu etkenler, laboratuvar sonuçları, komplikasyonlar ve tedavi seçeneklerinin gözden geçirilmesi amaçlandı.Gereç ve Yöntem: Uludağ Üniversitesi Tıp Fakültesi Dermatoloji Anabilim Dalı,Çocuk Nöroloji Bilim Dalı ve Çocuk Alerji Bilim Dalı’na 2000-2008 yılları arasında başvuran SJS ve TEN tanısı almış olan hastalar retrospektif olarak incelenmiştir.Bulgular: Toplam 14 hastanın 7’si kız ve 7’si erkek kayıtları incelendi. Hastaların 9’u SJS, 2’si SJS-TEN overlap sendrom, 3’ü TEN idi. Dokuz hastanın epilepsi tanısı ile, diğer hastaların anksiyete, diş apsesi, kriptik tonsillit, otitis media vebronşit tanıları ile ilaç kullanım öyküleri mevcuttu. Hastaların 11’inde %78,5 çoklu ilaç kullanımı bulunmaktaydı. SJS/TEN etyolojisinde 7 hastada %50 Lamotrijin 7 hastanın 4'ünde ek olarak Valproik asit kullanımı vardı diğer hastalarda Okskarbamazepin, Azitromisin, Fenitoin, İmipramin, Sefuroksim aksetil,Siprofloksasin, Seftriakson kullanımının sorumlu olduğu düşünüldü. Oniki hastaya sistemik kortikosteroid, 2 hastaya intravenöz immünglobulin tedavisi uygulanmıştı ve hastaların tümünde tam iyileşme görüldüğü saptandı.Sonuç: İlaç reaksiyonları çocuklarda erişkinlere oranla daha az görülmekle birlikte uzun süreli ve çoklu ilaç kullanımı olan çocuk hastalarda, SJS ve TENgibi şiddetli ilaç reaksiyonlarının gelişme riski göz önünde bulundurulmalıdır

Anahtar Kelimeler

• Steven’s Johnson Sendromu,toksik epidermal nekroliz,çocuk

Steven’s Johnson Syndrome and Toxic Epidermal Necrolysis in Children

Abstract

Aim: The aim of this study was to consider clinical features, laboratory findings, treatment alternatives, complications and responsible agents of Steven’s Johnson Syndrome SJS and Toxic Epidermal Necrolysis TEN in childhood. Materials and Method: The patients who were diagnosed with Steven’s Johnson syndrome SJS or Toxic Epidermal Necrolysis TEN and followed by Department of Dermatology, Division of Pediatric Neurology and Divisionof Pediatric Allergy of Uludag University Faculty of Medicine, between 2000-2008 were evaluated retrospectively.Results: Records of 14 patients 7 females/ 7 males were examined. Nine of thepatients had SJS, 2 had SJS-TEN overlap syndrome and the rest 3 had TEN. Ninepatients had history of using drugs for epilepsy and the others for anxiety, toothabscess, cryptic tonsillitis, otitis media and bronchiolitis. Eleven of the patients 78.5% had multiple drug intake. In 7 patients 50% Lamotrigine 4 of 7 patientswas also taken Valproic acid and in other patients oxcarbamazepine, azytromicine, phenytoin, imipramine, cefuroxime axetil, ciprofloxacin, ceftriaxone were responsible for SJS and TEN. Twelve patients were treated with systemic corticosteroids and 2 with intravenous immunoglobulin. All of the patients were cured. Conclusion: Although drug reactions were seen less frequently in children compared to adults, it must be taken into consideration that there is the risk of developing severe drug reactions like SJS and TEN in children who have been using such drugs for a long time or using multipl drugs for extended periods.

Keywords

• Steven’s Johnson Syndrome, Toxic Epidermal Necrolysis, children

Kaynakça

1. 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. 2. Forman R, Koren G, Shear NH. Erythema multiforme, Stevens-Johnson syndrome and toxic epidermal necrolysis in children: a review of 10 years' experience. Drug Safety 2002; 25: 965-72.
3. 3. Huff JC, Weston WL, Tonnesen MG. Erythema multiforme: a critical review of characteristics, diagnostic criteria and causes. J Am Acad Dermatol 1983; 8: 763-75.
4. 4. Shin HT, Change MW. Drug eruptions in children. Probl Dermatol 2002; 14: 147-82.
5. 5. Dore J, Salisbury RE. Morbidity and mortality of mucocutaneous diseases in the pediatric population at a tertiary care center. J Burn Care Res 2007; 28: 865-70.
6. 6. Friedmann PS, Leew MS, Friedmann AC, Barnetson RSC. Mechanisms in cutaneous drug hypersensitivity reactions. Clin Exp Allergy 2003; 33: 861-72.
7. 7. Fritsch PO, Sidoroff A. Drug-induced Stevens-Jonson syndrome/toxic epidermal necrolysis. Am J Clin Dermatol 2000; 1: 349-60.
8. 8. Paul C, Wolkenstein P, Adle H, et al. Apopitosis as a mechanism of keratinocyte death in toxic epidermal necrolysis. Br J Dermatol 1996; 134: 710-4.

9. 9. Letko E, Papaliodis DN, Papaliodis GN, Yasinse JD, Ahmed AR, Foster S. Stevens-Johnson syndrome and toxic epidermal necrolysis: A review of the literature. Ann Allergy Asthma Immunol 2005; 94: 419-36.
10. 10. Paquet P, Pierard GE. Erythema multiforme and toxic epidermal necrolysis: A comparative study. Am J Dermatopathol 1997; 19: 127-32.
11. 11. 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.
12. 12. Ruiz-Maldonado R. Acute disseminated epidermal necrosis types 1,2 and 3: Study of sixty cases. J Am Acad Dermatol 1985; 13: 623-35.
13. 13. Roujeau JC, Stern RS. Severe adverse cutaneous reactions to drugs. N Engl J Med 1994; 331: 1272-85.
14. 14. French EL, Prins C. Toxic Epidermal Necrolysis. In: Bolognia JL, Jorizzo JL, Rapini RP, editors. Dermatology. St Louis: Mosby; 2003. p. 323-31.
15. 15. Spies M, Sanford AP, Aili Low JF, Wolf SE, Herndon DN. Treatment of extensive toxic epidermal necrolysis in children. Pediatrics 2001; 108: 1162-8.
16. 16. Thomé-Souza S, Freitas A, Fiore LA, Valente KD. Lamotrigine and valproate: efficacy of co-administration in a pediatric population. Pediatr Neurol 2003; 28: 360-4.
17. 17. 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.
18. 18. Roujeau JC. The spectrum of Stevens-Johnson syndrome and toxic epidermal necrolysis: A clinical classification. J Invest Dermatol 1994; 102: 28-30.
19. 19. Roujeau JC, Huynh TN, Bracq C, Guillaume JC, Revuz J, Touraine R. Genetic susceptibility to toxic epidermal necrolysis. Arch Dermatol 1987; 123: 1171-3.
20. 20. Akman A, Alpsoy E. Eritema Multiforme, Steven-Johnson Sendromu ve Toxic Epidermal Nekrolizis (Lyell Sendromu). Türkiye Klinikleri J Surg Med Sci 2006; 2: 6-15.
21. 21. Fritsch PO, Sidoroff A. Drug- induced Stevens-Johnson syndrome/toxic epidermal necrolysis. Am J Clin Dermatol 2000; 1: 349-60.
22. 22. Schöpf E, Stühmer 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.
23. 23. Revuz J, Penso D, Roujeau JC, Guillaume JC, Payne CR, Wechsler J, Touraine R. Toxic epidermal necrolysis. Clinical findings and prognosis factors in 87 patients. Arch Dermatol 1987; 123: 1160-5.
24. 24. 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;14; 333: 1600-7.
25. 25. Wallis C, McClymont W. Toxic epidermal necrolysis with adult respiratory distress sydrome. Anaesthesia 1995;50:801-3.
26. 26. Avakian R, Flowers FP, Araujo OE, Ramos-Caro FA. Toxic epidermal necrolysis: A review. J Am Acad Dermatol 1991; 25: 69-79.
27. 27. Rasmussen JE. Erythema multiforme. Should anyone care about the standards of care? Arch Dermatol 1995; 131: 726-9.
28. 28. Adzick NS, Kim SH, Bondoc CC, Quinby WC, Remensnyder JP. Management of toxic epidermal necrolysis in a pediatric burn center. Am J Dis Child 1985; 139: 499-502.
29. 29. Murphy JT, Purdue GF, Hunt JL. Toxic epidermal necrolysis. J Burn Care Rehabil 1997; 18: 417-20.
30. 30. Kakourou T, Klontza D, Soteropoulou F, Kattamis C. Corticosteroid treatment of erythema multiforme major (StevensJohnson syndrome) in children. Eur J Pediatr 1997; 156: 90-3.
31. 31. Criton S, Devi K, Sridevi PK, Asokan PU. Toxic epidermal necrolysis-a retrospective study. Int J Dermatol 1997; 36: 923-5.
32. 32. Tripathi A, Ditto AM, Grammer LC, Greenberger PA, McGrath KG, Zeiss CR, et al. Corticosteroid therapy in an additional 13 cases of Stevens-Johnson syndrome: a total series of 67 cases. Allergy Asthma Proc 2000; 21: 101-5.
33. 33. Halebian PH, Corder VJ, Madden MR, Finkelstein JL, Shires GT. Improved burn center survival of patients with toxic epidermal necrolysis managed without corticosteroids. Ann Surg 1986; 204: 503-12.
34. 34. Jones WG, Halebian P, Madden M, Finkelstein J, Goodwin CW. Drug induced toxic epidermal necrolysis in children. J Pediatr Surg 1989; 24: 167-70.
35. 35. Magina S, Lisboa C, Goncalves E, Conceicao F, Leal V, Mesquita-Guimaraes J. A case of toxic epidermal necrolysis treated with intravenous immunglobulin. Br J Dermatol 2000; 142: 191-2.
36. 36. Chaidemenos GC, Chrysomallis F, Sombolos K, Mourellou O, Ioannides D, Papakonstantinou M. Plasmapheresis in toxic epidermal necrolysis. Int J Dermatol 1997; 36: 218-21.
37. 37. Heng MC, Allen SG. Efficacy of cyclophosphamide in toxic epidermal necrolysis. Clinical and pathophysiologic aspects. J Am Acad Dermatol 1991; 25: 778-86.
38. 38. Renfro L, Grant-Kels JM, Daman LA. Drug-induced toxic epidermal necrolysis treated with cyclosporin. Int J Dermatol 1989; 28: 441-4.
39. 39. Furubacke A, Berlin G, Anderson C, Sjoberg F. Lack of significant treatment effect of plasma Exchange in the treatment of drug induced toxic epidermal necrolysis. Intensive Care Med 1999; 25:1307-10.
40. 40. Trautmann A, Klein CE, Kampgen E, Brocker EB. Severe bullous drug reactions treated succesfully with cyclophosphamide. Br J Dermatol 1998; 139: 1127-8.
41. 41. Arevalo JM, Lorente JA, Gonzalez-Herrada C, Jimenez-Reyes J. Treatment of toxic epidermal necrolysis with cyclosporin. A J Trauma 2000; 48: 473-8.