A SYSTEMATIC REVIEW OF TREATMENT OF DRUG-INDUCED STEVENS-JOHNSON SYNDROME AND TOXIC EPIDERMAL NECROLYSIS IN CHILDREN

Main Article Content

Blanca R Del Pozzo-Magana
Alejandro Lazo-Langner
Bruce Carleton
Lucila I Castro-Pastrana
Michael J Rieder

Keywords

Stevens-Johnson syndrome, toxic epidermal necrolysis, children, drugs, systematic review

Abstract

Stevens-Johnson (SJS) and Toxic Epidermal Necrolysis (TEN) are two uncommon mucocutaneous diseases usually considered as severe drug reactions and are characterized by different grades of epidermal necrosis. Several treatment modalities have been proposed with variable results but the lack of controlled studies makes difficult to analyze them objectively especially in children. All publications describing management for SJS and TEN in children were searched in MEDLINE, EMBASE, and the Cochrane Library. Reports included were divided in two categories: A, studies with 5 or more patients and observational studies; and B, reports with less than 5 patients. A formal meta-analysis was not feasible. Description was made using central tendency measures. From 1389 references only 31 references with a total of 128 cases were included, 88 category A and 40 category B. The 4 main treatment modalities were: intravenous immunoglobulin (IVIG), steroids (prednisolone, methylprednisolone, dexamethasone), dressings with or without surgical debridement, and support treatment alone. Miscellaneous treatments: Of 12 patients, 3 received ulinastatin, 4 patients plasmapheresis, 2 patients IV pentoxifylline and the last three patients received different treatment each (cyclosporine, methylprednisone/G-CSF and methylprednisolone/IVIG). Patients receiving IVIG and steroids showed similar findings while patients treated with dressing and support treatment alone, reported both longer time to achieve remission and hospitalization stays and appear to be associated with more complications and deaths. There is scant quality literature about management of SJS and TEN in children. Steroids and IVIG seem to improve the outcome of SJS and TEN patients but results from different reports are variable. Patients treated only with care support seem to have higher morbidity and mortality. Further studies are necessary to define optimal management.

Abstract 592 | PDF Downloads 287

References

1. Strom BL, Carson L, Halpern AC, Schinnar R, Snyder ES, Tilson HH, et al. A population-based study of Stevens-Johnson syndrome. Incidence and antecedent drug exposures. Arch Dermatol 1991; 127(6):831-8.
2. 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.
3. Roujeau JC, Stern RS. Severe adverse cutaneous reactions to drugs. N Eng J Med 1994; 331(19):1272-85.
4. Klein PA. Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis. E-medicine 2006 Dec 4; Available from: http://www.emedicine.com/derm/TOPIC405.HTM . Accessed June 3, 2009.
5. Knowles SR, Shapiro LE, Shear NH. Drug eruptions. In: Schachner LA, Hansen RC, editors. Pediatric Dermatology. 3rd Edition ed. London, UK: Mosby; 2003. p. 1267-76.
6. Weston WL, Orchard D. Vascular reactions. In: Schachner LA, Hansen RC, editors. Pediatric Dermatology. 3rd Edition ed. London, UK: Mosby; 2003. p. 801-31.
7. Mittmann N, Chan BC, Knowles S, Shear NH. IVIG for the treatment of toxic epidermal necrolysis. Skin Therapy Lett 2007 Feb;12(1):7-9.
8. Sehgal VN, Srivastava G. Toxic epidermal necrolysis (TEN) Lyell's syndrome. J Dermatol Treat 2005;16(5-6):278-86.
9. Bastuji-Garin S, Razny B, Stern RS, Shear H, Naldi L, Roujeau J. Clinical classification of cases of toxic epidermal necrolysis, Stevens- Johnson syndrome, and erythema multiforme. Arch Dermatol 1993;129(1):92-6.
10. Forman R, Koren G, Shear NH. Erythema multiforme, Stevens-Johnson syndrome and toxic epidermal necrolysis in children: a review of 10 years' experience. Drug Saf 2002; 25(13):965-72.
11. Allanore L, Roujeau J-C. Clinic and pathogenesis of severe bullous skin reactions: Stevens-Johnson syndrome, toxic epidermal necrolysis. In: Pichler WJ, editor. Drug hyepersensitivity. 1st ed. Basel: Karger; 2007. p. 267-77.
12. Ruiz-Maldonado R. Acute disseminated epidermal necrosis types 1, 2, and 3: study of sixty cases. J Am Acad Dermatol 1985;13(4):623-35.
13. Edell DS, Muelenaer AA, Majure M, Davidson JJ. Unusual manifestation of Stevens-Johnson syndrome involving the respiratory and gastrointestinal tract. Pediatrics 1992;89(3):429- 32.
14. Prendeville JS. Erythema multiforme and Stevens-Johnson syndrome in children. Curr Opin Pediatr 1991;3:673-7.
15. Guegan S, Bastuji-Garin S, Poszepcznska- Guigne E, Roujeau J, Revuz J. 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.
16. Trent J, Kirsner RS, Romanelli P, Kerdel FA. Analysis of intravenous Immunoglobulin for the Treatment of Toxic epidermal Necrolysis using SCORTEN. Arch Dermatol 2003;139:39-43.
17. Garcia D, I, Le Cleach L, Bocquet H, Otero XL, Roujeau J. Toxic epidermal necrolysis and Stevens-Johnson syndrome. Does early withdrawal of causative drugs decrease the risk of death? Arch Dermatol 2000; 136(March):323-7.
18. Smith ML. Pediatric burns: management of thermal, electrical, and chemical burns and burnlike dermatologic conditions. Pediatr Ann 2000 Jun;29(6):367-78.
19. Ramakrishnan KM, Sankar J, Venkatraman J. Role of biological membranes in the management of Stevens Johnson syndrome--Indian experience. Burns 2007 Feb; 33(1):109-11.
20. Ringheanu M, Laude TA. Toxic epidermal necrolysis in children--an update. Clin Pediatr (Phila) 2000 Dec;39(12):687-94.
21. Chave TA, Mortimer NJ, Sladden MJ, Hall AP, Hutchinson PE. Toxic epidermal necrolysis: current evidence, practical management and future directions. Br J Dermatol 2005 Aug;153(2):241-53.
22. Ghislain PD, Roujeau JC. Treatment of severe drug reactions: Stevens-Johnson syndrome, toxic epidermal necrolysis and hypersensitivity syndrome. Dermatol Online J 2002 Jun;8(1):5.
23. Yeung A, Goldman R. Use of steroids for erythema multiforme in children. Can Fam Physician 2005;51(11):1481-3.
24. Hynes AY, Kafkala C, Daoud YJ, Foster CS. Controversy in the use of high-dose systemic steroids in the acute care of patients with Stevens-Johnson syndrome. Int Ophthalmol Clin 2005;45(4): 25-48.
25. 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 Oct 16; 282(5388):490-3.
26. Prins C, Kerdel FA, Padilla RS, Hunziker T, Chimenti S, Viard I, et al. Treatment of toxic epidermal necrolysis with high-dose intravenous immunoglobulins: multicenter retrospective analysis of 48 consecutive cases. Arch Dermatol 2003 Jan;139(1):26-32.
27. Bahna SL, Khalili B. New concepts in the management of adverse drug reactions. Allergy Asthma Proc 2007 Sep;28(5):517-24.
28. Khalili B, Bahna SL. Pathogenesis and recent therapeutic trends in Stevens-Johnson syndrome and toxic epidermal necrolysis. Ann Allergy Asthma Immunol 2006 Sep;97(3):272-80.
29. Majumdar S, Mockenhaupt M, Roujeau J, Townshend A. Interventions for toxic epidermal necrolysis. Cochrane Database Syst Rev 2002;(4):CD001435.
30. Vandenbroucke JP, von EE, Altman DG, Gotzsche PC, Mulrow CD, Pocock SJ, et al. Strengthening the Reporting of Observational Studies in Epidemiology (STROBE): explanation and elaboration. PLoS Med 2007 Oct 16;4(10):e297.
31. von EE, Altman DG, Egger M, Pocock SJ, Gotzsche PC, Vandenbroucke JP. The Strengthening the Reporting of Observational Studies in Epidemiology (STROBE) statement: guidelines for reporting observational studies. PLoS Med 2007 Oct 16;4(10):e296.
32. Kakourou T, Klontza D, Soteropoulou F, Kattamis C. Corticosteroid treatment of erythema multiforme major (Stevens-Johnson syndrome) in children. Eur J Pediatr 1997 Feb;156(2):90-3.
33. Al-Mutairi N, Arun J, Osama NE, Amr Z, Mazen AS, Ibtesam EA, et al. Prospective, noncomparative open study from Kuwait of the role of intravenous immunoglobulin in the treatment of toxic epidermal necrolysis. Int J Dermatol 2004 Nov 30;43(11):847-51.
34. Chaidemenos GC, Chrysomallis F, Sombolos K, Mourellou O, Ioannides D, Papakonstantinou M. Plasmapheresis in toxic epidermal necrolysis. Int J Dermatol 1997 Mar;36(3):218-21.
35. Egan CA, Grant WJ, Morris SE, Saffle JR, Zone JJ. Plasmapheresis as an adjunct treatment in toxic epidermal necrolysis. J Am Acad Dermatol 1999 Mar;40(3):458-61.
36. Inamo Y, Okubo T, Wada M, Fuchigami S, Hashimoto K, Fuchigami T, et al. Intravenous ulinastatin therapy for Stevens-Johnson syndrome and toxic epidermal necrolysis in pediatric patients. Three case reports. Int Arch Allergy Immunol 2002 Jan;127(1):89-94.
37. Mangla K, Rastogi S, Goyal P, Solanki RB, Rawal RC. Efficacy of low dose intravenous immunoglobulins in children with toxic epidermal necrolysis: an open uncontrolled study. Indian J Dermatol Venereol Leprol 2005 Nov;71(6):398-400.
38. 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(4): 347-52.
39. Morici MV, Galen WK, Shetty AK, Lebouef RP, Gouri TP, Cowan GS, et al. Intravenous immunoglobulin therapy for children with Stevens-Johnson syndrome. J Rheumatol 2000 Oct;27(10):2494-7.
40. Metry DW, Jung P, Levy ML. Use of intravenous immunoglobulin in children with stevens-johnson syndrome and toxic epidermal necrolysis: seven cases and review of the literature. Pediatrics 2003 Dec;112(6 Pt 1):1430- 6.
41. Tristani-Firouzi P, Petersen MJ, Saffle JR, Morris SE, Zone JJ. Treatment of toxic epidermal necrolysis with intravenous immunoglobulin in children. J Am Acad Dermatol 2002 Oct;47(4):548-52.
42. Sheridan RL, Weber JM, Schulz JT, Ryan CM, Low HM, Tompkins RG. Management of severe toxic epidermal necrolysis in children. J Burn Care Rehabil 1999 Nov;20(6):497-500.
43. Aihara Y, Ito R, Ito S, Aihara M, Yokota S. Toxic epidermal necrolysis in a child successfully treated with cyclosporin A and methylprednisolone. Pediatr Int 2007 Oct;49(5):659-62.
44. Arca E, Kose O, Erbil AH, Nisanci M, Akar A, Gur AR. A 2-year-old girl with Stevens-- Johnson syndrome/toxic epidermal necrolysis treated with intravenous immunoglobulin. Pediatr Dermatol 2005 Jul;22(4):317-20.
45. Asz J, Asz D, Moushey R, Seigel J, Mallory SB, Foglia RP. Treatment of toxic epidermal necrolysis in a pediatric patient with a nanocrystalline silver dressing. J Pediatr Surg 2006 Dec;41(12): e9-12.
46. Bannasch H, Kontny U, Kruger M, Stark GB, Niemeyer CM, Brandis M, et al. A semisynthetic bilaminar skin substitute used to treat pediatric full-body toxic epidermal necrolysis: wraparound technique in a 17- month-old girl. Arch Dermatol 2004 Feb;140(2):160-2.
47. Bott L, Santos C, Thumerelle C, Mars A, Deschildre A, Catteau B. Syndrome de Stevens- Johnson sévère chez l'enfant: à propos de 4 observations. Arch Pediatr 2007;14(12):1435-8.
48. Bygum A, Gregersen JW, Buus SK. Acetaminophen-induced toxic epidermal necrolysis in a child. Pediatr Dermatol 2004 May;21(3):236-8.
49. (49) Cazzola G, Nicolussi M, Carraro F. High-dose i.v. 7S immunoglobulin treatment in Stevens-Johnson syndrome. Helv Paediatr Acta 1986;41(1-2):87-8.
50. de Juan Martin F, Bouthelier Moreno M, Marin Bravo MC, Melendo Gimeno J. Necrolisis epidérmica tóxica tratada con inmunoglobulina intravenosa. An Esp Pediatr 2002 Apr;56(4):370-1.
51. Kalyoncu M, Cimsit G, Cakir M, Okten A. Toxic epidermal necrolysis treated with intravenous immunoglobulin and granulocyte colony-stimulating factor. Indian Pediatr 2004 Apr;41(4):392-5.
52. Kuhn-Cordova I, Ramirez-Bouchan D, Gamboa- Marrufo JD. Uso de inmunoglobulina intravenosa en el tratamiento de necrolisis epidérmica tóxica y síndrome de Stevens-Johnson. An Pediatr (Barc ) 2007 Jul;67(1):68-73.
53. Lehrer-Bell KA, Kirsner RS, Tallman PG, Kerdel FA. Treatment of the cutaneous involvement in Stevens-Johnson syndrome and toxic epidermal necrolysis with silver nitrateimpregnated dressings. Arch Dermatol 1998 Jul;134(7):877-9.
54. Mayorga C, Torres MJ, Corzo JL, Sanchez- Sabate E, Alvarez J, Vera A, et al. Improvement of toxic epidermal necrolysis after the early administration of a single high dose of intravenous immunoglobulin. Ann Allergy Asthma Immunol 2003 Jul;91(1):86-91.
55. Munoz Romero F, Mallent Anon J, Laredo Ortiz C, Domenech Miro E, Tafalla Navarro M, Tafalla Pena M. Necrolisis epidérmica tóxica: presentación de dos casos pediátricos. An Esp Pediatr 1996 Jul;45(1):71-5.
56. Pasic S. Intravenous immunoglobulin in toxic epidermal necrolysis. Int J Dermatol 2006 Sep;45(9):1117-8.
57. Sanclemente G, De la Roche CA, Escobar CE, Falabella R. Pentoxyfylline in toxic epidermal necrolysis and Stevens-Johnson syndrome. Int J Dermatol 1999 Nov;38(11):878-9.
58. Zipitis CS, Thalange N. Intravenous immunoglobulins for the management of Stevens-Johnson syndrome with minimal skin manifestations. Eur J Pediatr 2007 Jun;166(6):585-8.

Most read articles by the same author(s)