The Korean journal of internal medicine

The Korean Association of Internal Medicine (대한내과학회)
권호 표지
DOI QR코드

DOI QR Code

Causes and Treatment Outcomes of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis in 82 Adult Patients

  • Kim, Hye-In (Department of Internal Medicine, Kyungpook National University School of Medicine) ;
  • Kim, Shin-Woo (Department of Internal Medicine, Kyungpook National University School of Medicine) ;
  • Park, Ga-Young (Department of Internal Medicine, Kyungpook National University School of Medicine) ;
  • Kwon, Eu-Gene (Department of Internal Medicine, Kyungpook National University School of Medicine) ;
  • Kim, Hyo-Hoon (Department of Internal Medicine, Kyungpook National University School of Medicine) ;
  • Jeong, Ju-Young (Department of Internal Medicine, Kyungpook National University School of Medicine) ;
  • Chang, Hyun-Ha (Department of Internal Medicine, Kyungpook National University School of Medicine) ;
  • Lee, Jong-Myung (Department of Internal Medicine, Kyungpook National University School of Medicine) ;
  • Kim, Neung-Su (Department of Internal Medicine, Kyungpook National University School of Medicine)
  • Published : 2012.06.01
⟨ Previous Next ⟩

Abstract

Background/Aims: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are predominantly known as medication-induced diseases. However, at our institution, we have experienced more cases of non-drug-related SJS and TEN than expected. Therefore, we studied the difference between non-drug-related and drug-related SJS and TEN in terms of clinical characteristics and prognoses. Methods: The etiologies, clinical characteristics, and treatment outcomes for 82 adult patients with SJS and TEN were retrospectively reviewed. Results: A total of 71 patients (86.6%) were classified as having SJS, and the other 11 patients (13.4%) were classified as having TEN. Drug-related cases were more common (43, 52.4%) than non-drug-related cases (39, 47.6%). Anticonvulsants (12/82, 14.6%) and antibiotics (9/82, 11%) were the most common causative medications. Anemia (p = 0.017) and C-reactive protein of ${\geq}$ 5 mg/dL (p = 0.026) were more common in the drug-related cases than in the nondrug- related cases. Intravenous steroid therapy was used as the main treatment regimen (70/82, 85.4%). Of the 82 patients, 8 (9.8%) died during the clinical course. A univariate analysis for mortality showed statistical significance for the following: kidney function abnormality, pneumonia, hemoglobin of < 10 g/dL, and combined underlying diseases. In a multivariate analysis, only pneumonia was statistically significant (odds ratio, 25.79; p = 0.009). Conclusions: Drugs were the most frequent cause of these diseases. However, non-drug-related causes also contributed to a significant proportion of cases. Physicians should keep this in mind when documenting patient history. In addition, early recognition and treatment may be important for better outcomes.

Keywords

References

  1. Borchers AT, Lee JL, Naguwa SM, Cheema GS, Gershwin ME. Stevens-Johnson syndrome and toxic epidermal necrolysis. Autoimmun Rev 2008;7:598-605. https://doi.org/10.1016/j.autrev.2008.06.004
  2. Mockenhaupt M. The current understanding of Stevens-Johnson syndrome and toxic epidermal necrolysis. Expert Rev Clin Immunol 2011;7:803-813. https://doi.org/10.1586/eci.11.66
  3. Bachot N, Roujeau JC. Differential diagnosis of severe cutaneous drug eruptions. Am J Clin Dermatol 2003;4:561-572. https://doi.org/10.2165/00128071-200304080-00006
  4. Auquier-Dunant A, Mockenhaupt M, Naldi L, et al. Correlations between clinical patterns and causes of erythema multiforme majus, Stevens-Johnson syndrome, and toxic epidermal necrolysis: results of an international prospective study. Arch Dermatol 2002;138:1019-1024. https://doi.org/10.1001/archderm.138.8.1019
  5. Tay YK, Huff JC, Weston WL. Mycoplasma pneumoniae infection is associated with Stevens-Johnson syndrome, not erythema multiforme (von Hebra). J Am Acad Dermatol 1996;35(5 Pt 1):757-760. https://doi.org/10.1016/S0190-9622(96)90732-X
  6. Mulvey JM, Padowitz A, Lindley-Jones M, Nickels R. Mycoplasma pneumonia associated with Stevens Johnson syndrome. Anaesth Intensive Care 2007;35:414-417.
  7. Leigh IM, Mowbray JF, Levene GM, Sutherland S. Recurrent and continuous erythema multiforme: a clinical and immunological study. Clin Exp Dermatol 1985;10:58-67. https://doi.org/10.1111/j.1365-2230.1985.tb02553.x
  8. Bastuji-Garin S, Fouchard N, Bertocchi M, Roujeau JC, Revuz J, Wolkenstein P. SCORTEN: a severity-of-illness score for toxic epidermal necrolysis. J Invest Dermatol 2000;115:149-153. https://doi.org/10.1046/j.1523-1747.2000.00061.x
  9. Schneck J, Fagot JP, Sekula P, Sassolas B, Roujeau JC, Mockenhaupt M. 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. https://doi.org/10.1016/j.jaad.2007.08.039
  10. Power WJ, Ghoraishi M, Merayo-Lloves J, Neves RA, Foster CS. Analysis of the acute ophthalmic manifestations of the erythema multiforme/Stevens-Johnson syndrome/toxic epidermal necrolysis disease spectrum. Ophthalmology 1995;102:1669-1676. https://doi.org/10.1016/S0161-6420(95)30811-1
  11. 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;129:92-96. https://doi.org/10.1001/archderm.1993.01680220104023
  12. Rzany B, Mockenhaupt M, Baur S, et al. Epidemiology of erythema exsudativum multiforme majus, Stevens-Johnson syndrome, and toxic epidermal necrolysis in Germany (1990- 1992): structure and results of a population-based registry. J Clin Epidemiol 1996;49:769-773. https://doi.org/10.1016/0895-4356(96)00035-2
  13. Chang YS, Huang FC, Tseng SH, Hsu CK, Ho CL, Sheu HM. Erythema multiforme, Stevens-Johnson syndrome, and toxic epidermal necrolysis: acute ocular manifestations, causes, and management. Cornea 2007;26:123-129. https://doi.org/10.1097/ICO.0b013e31802eb264
  14. Kim EJ, Lee JB, Kwon YH, et al. A clinical study of Stevens- Johnson syndrome and toxic epidermal necrolysis over the last five-year period in the Gwangju-Chonnam area. Korean J Dermatol 2006;44:574-578.
  15. Mockenhaupt M, Viboud C, Dunant A, et al. Stevens-Johnson syndrome and toxic epidermal necrolysis: assessment of medication risks with emphasis on recently marketed drugs. The EuroSCAR-study. J Invest Dermatol 2008;128:35-44. https://doi.org/10.1038/sj.jid.5701033
  16. Jeung YJ, Lee JY, Oh MJ, Choi DC, Lee BJ. Comparison of the causes and clinical features of drug rash with eosinophilia and systemic symptoms and stevens-johnson syndrome. Allergy Asthma Immunol Res 2010;2:123-126. https://doi.org/10.4168/aair.2010.2.2.123
  17. Wetter DA, Camilleri MJ. Clinical, etiologic, and histopathologic features of Stevens-Johnson syndrome during an 8-year period at Mayo Clinic. Mayo Clin Proc 2010;85:131-138. https://doi.org/10.4065/mcp.2009.0379
  18. Revuz J, Penso D, Roujeau JC, et al. Toxic epidermal necrolysis: clinical findings and prognosis factors in 87 patients. Arch Dermatol 1987;123:1160-1165. https://doi.org/10.1001/archderm.1987.01660330071012
  19. Correia O, Chosidow O, Saiag P, Bastuji-Garin S, Revuz J, Roujeau JC. Evolving pattern of drug-induced toxic epidermal necrolysis. Dermatology 1993;186:32-37. https://doi.org/10.1159/000247299
  20. Stewart MG, Duncan NO 3rd, Franklin DJ, Friedman EM, Sulek M. Head and neck manifestations of erythema multiforme in children. Otolaryngol Head Neck Surg 1994;111(3 Pt 1):236-242.
  21. Khoo AK, Foo CL. Toxic epidermal necrolysis in a burns centre: a 6-year review. Burns 1996;22:275-278. https://doi.org/10.1016/0305-4179(95)00140-9
  22. Pushker N, Tandon R, Vajpayee RB. Stevens-Johnson syndrome in India-risk factors, ocular manifestations and management. Ophthalmologica 2000;214:285-288. https://doi.org/10.1159/000027505
  23. French LE. Toxic epidermal necrolysis and Stevens Johnson syndrome: our current understanding. Allergol Int 2006;55:9-16. https://doi.org/10.2332/allergolint.55.9
  24. Pereira FA, Mudgil AV, Rosmarin DM. Toxic epidermal necrolysis. J Am Acad Dermatol 2007;56:181-200. https://doi.org/10.1016/j.jaad.2006.04.048
  25. Kardaun SH, Jonkman MF. Dexamethasone pulse therapy for Stevens-Johnson syndrome/toxic epidermal necrolysis. Acta Derm Venereol 2007;87:144-148. https://doi.org/10.2340/00015555-0214
  26. Araki Y, Sotozono C, Inatomi T, et al. Successful treatment of Stevens-Johnson syndrome with steroid pulse therapy at disease onset. Am J Ophthalmol 2009;147:1004-1011. https://doi.org/10.1016/j.ajo.2008.12.040
  27. Faye O, Roujeau JC. Treatment of epidermal necrolysis with high-dose intravenous immunoglobulins (IV Ig): clinical experience to date. Drugs 2005;65:2085-2090. https://doi.org/10.2165/00003495-200565150-00002
  28. Brown KM, Silver GM, Halerz M, Walaszek P, Sandroni A, Gamelli RL. Toxic epidermal necrolysis: does immunoglobulin make a difference? J Burn Care Rehabil 2004;25:81-88. https://doi.org/10.1097/01.BCR.0000105096.93526.27
  29. Shortt R, Gomez M, Mittman N, Cartotto R. Intravenous immunoglobulin does not improve outcome in toxic epidermal necrolysis. J Burn Care Rehabil 2004;25:246-255. https://doi.org/10.1097/01.BCR.0000124746.33279.86

Cited by

  1. Rapidly Developing Toxic Epidermal Necrolysis vol.2013, pp.None, 2012, https://doi.org/10.1155/2013/985951
  2. Stevens-Johnson syndrome / toxic epidermal necrolysis: an Asia-Pacific perspective vol.3, pp.4, 2013, https://doi.org/10.5415/apallergy.2013.3.4.215
  3. Chronic pulmonary complications due to toxic epidermal necrolysis vol.1, pp.4, 2012, https://doi.org/10.4168/aard.2013.1.4.391
  4. Retrospective Analysis of Corticosteroid Treatment in Stevens-Johnson Syndrome and/or Toxic Epidermal Necrolysis over a Period of 10 Years in Vajira Hospital, Navamindradhiraj University, Bangkok vol.2014, pp.None, 2012, https://doi.org/10.1155/2014/237821
  5. Therapeutic Apheresis in Dermatological Diseases vol.7, pp.None, 2012, https://doi.org/10.4137/cmt.s14112
  6. Corticosteroids in Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis : Current Evidence and Implications for Future Research vol.49, pp.3, 2015, https://doi.org/10.1177/1060028014560012
  7. Stevens–Johnson syndrome following cataract surgery vol.5, pp.2, 2012, https://doi.org/10.1016/j.tjo.2014.05.005
  8. Stevens–Johnson syndrome and toxic epidermal necrolysis: a 20‐year single‐center experience vol.54, pp.8, 2012, https://doi.org/10.1111/ijd.12702
  9. Stevens-Johnson syndrome and toxic epidermal necrolysis in Dr. Hasan Sadikin General Hospital Bandung, Indonesia from 2009-2013 vol.6, pp.1, 2012, https://doi.org/10.5415/apallergy.2016.6.1.43
  10. Recurrent Toxic Epidermal Necrolysis Syndrome: A Report of Two Cases vol.3, pp.None, 2012, https://doi.org/10.1007/s40800-016-0032-x
  11. Retrospective Analysis of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis in 88 Chinese Patients vol.130, pp.9, 2012, https://doi.org/10.4103/0366-6999.204929
  12. Experience of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis in a Regional Hospital: 70 Cases in 7 Years vol.92, pp.2, 2012, https://doi.org/10.3904/kjm.2017.92.2.171
  13. Current Perspectives on Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis vol.54, pp.1, 2012, https://doi.org/10.1007/s12016-017-8654-z
  14. Clinical Features and Treatment Outcomes among Children with Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: A 20-Year Study in a Tertiary Referral Hospital vol.2018, pp.None, 2018, https://doi.org/10.1155/2018/3061084
  15. The Epidemiology of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis in China vol.2018, pp.None, 2012, https://doi.org/10.1155/2018/4320195
  16. DILI Associated with Skin Reactions vol.17, pp.3, 2012, https://doi.org/10.1007/s11901-018-0414-x
  17. Lip synechiae: A rare complication of azithromycin-associated Stevens-Johnson syndrome vol.10, pp.2, 2019, https://doi.org/10.4103/njms.njms_24_19
  18. Diagnostic and Treatment Approach in the Management of Dental Anomalies Associated with Stevens-Johnson Syndrome: A Case Report vol.14, pp.4, 2021, https://doi.org/10.5005/jp-journals-10005-1986
  19. Viral Infections and Cutaneous Drug-Related Eruptions vol.11, pp.None, 2012, https://doi.org/10.3389/fphar.2020.586407
  20. Sex Differences in the Risk of Cutaneous Adverse Drug Reactions Induced by Antiseizure Medications: A Systematic Review and Meta-analysis vol.35, pp.2, 2021, https://doi.org/10.1007/s40263-021-00794-0