Skip to main content
PMC home page
Archives of Disease in Childhood logoLink to Archives of Disease in Childhood
. 2000 Oct;83(4):347–352. doi: 10.1136/adc.83.4.347

Diagnosis, classification, and management of erythema multiforme and Stevens-Johnson syndrome

C Leaute-Labreze 1, T Lamireau 1, D Chawki 1, J Maleville 1, A Taieb 1
PMCID: PMC1718505  PMID: 10999875

Abstract

BACKGROUND—In adults, erythema multiforme (EM) is thought to be mainly related to herpes infection and Stevens-Johnson syndrome (SJS) to drug reactions.
AIMS—To investigate this hypothesis in children, and to review our experience in the management of these patients.
METHODS—A retrospective analysis of 77 paediatric cases of EM or SJS admitted to the Children's Hospital in Bordeaux between 1974and 1998.
RESULTS—Thirty five cases, inadequately documented or misdiagnosed mostly as urticarias or non-EM drug reactions were excluded. Among the remaining 42 patients (14 girls and 28 boys), 22 had EM (11EM minor and 11 EM major), 17 had SJS, and three had isolated mucous membrane involvement and were classified separately. Childhood EM was mostly related to herpes infection and SJS to infectious agents, especially Mycoplasma pneumoniae. Only two cases were firmly attributed to drugs (antibiotics). No patient died. EM and SJS sequelae were minor and steroids were of no overall benefit.
CONCLUSION—In paediatric practice EM is frequently misdiagnosed. The proposal that SJS is drug related in adults does not apply to children, and in our recruitment EM and SJS are mostly triggered by infectious agents. The course of both diseases, even though dramatic at onset, leads to low morbidity and mortality when appropriate symptomatic treatment is given.



Full Text

The Full Text of this article is available as a PDF (217.3 KB).

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Adzick N. S., Kim S. H., Bondoc C. C., Quinby W. C., Remensnyder J. P. Management of toxic epidermal necrolysis in a pediatric burn center. Am J Dis Child. 1985 May;139(5):499–502. doi: 10.1001/archpedi.1985.02140070073037. [DOI] [PubMed] [Google Scholar]
  2. Assier H., Bastuji-Garin S., Revuz J., Roujeau J. C. Erythema multiforme with mucous membrane involvement and Stevens-Johnson syndrome are clinically different disorders with distinct causes. Arch Dermatol. 1995 May;131(5):539–543. [PubMed] [Google Scholar]
  3. Bahmer F. A., Zaun H., Luszpinski P. Thalidomide treatment of recurrent erythema multiforme. Acta Derm Venereol. 1982;62(5):449–450. [PubMed] [Google Scholar]
  4. Barone C. M., Bianchi M. A., Lee B., Mitra A. Treatment of toxic epidermal necrolysis and Stevens-Johnson syndrome in children. J Oral Maxillofac Surg. 1993 Mar;51(3):264–268. doi: 10.1016/s0278-2391(10)80171-5. [DOI] [PubMed] [Google Scholar]
  5. Bastuji-Garin S., Rzany B., Stern R. S., Shear N. H., Naldi L., Roujeau J. C. Clinical classification of cases of toxic epidermal necrolysis, Stevens-Johnson syndrome, and erythema multiforme. Arch Dermatol. 1993 Jan;129(1):92–96. [PubMed] [Google Scholar]
  6. Brice S. L., Krzemien D., Weston W. L., Huff J. C. Detection of herpes simplex virus DNA in cutaneous lesions of erythema multiforme. J Invest Dermatol. 1989 Jul;93(1):183–187. doi: 10.1111/1523-1747.ep12277397. [DOI] [PubMed] [Google Scholar]
  7. Chan H. L., Stern R. S., Arndt K. A., Langlois J., Jick S. S., Jick H., Walker A. M. 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 Jan;126(1):43–47. [PubMed] [Google Scholar]
  8. Corticosteroids for erythema multiforme? Pediatr Dermatol. 1989 Sep;6(3):229–250. doi: 10.1111/j.1525-1470.1989.tb00824.x. [DOI] [PubMed] [Google Scholar]
  9. Di Lernia V., Lo Scocco G., Bisighini G. Erythema multiforme following hepatitis B vaccine. Pediatr Dermatol. 1994 Dec;11(4):363–364. doi: 10.1111/j.1525-1470.1994.tb00109.x. [DOI] [PubMed] [Google Scholar]
  10. Dikland W. J., Oranje A. P., Stolz E., van Joost T. Erythema multiforme in childhood and early infancy. Pediatr Dermatol. 1986 Feb;3(2):135–139. doi: 10.1111/j.1525-1470.1986.tb00504.x. [DOI] [PubMed] [Google Scholar]
  11. Ferrando M. F., Léauté-Labrèze C., Fleury H., Taïeb A. Orf and erythema multiforme in a child. Pediatr Dermatol. 1997 Mar-Apr;14(2):154–155. doi: 10.1111/j.1525-1470.1997.tb00226.x. [DOI] [PubMed] [Google Scholar]
  12. Ginsburg C. M. Stevens-Johnson syndrome in children. Pediatr Infect Dis. 1982 May-Jun;1(3):155–158. doi: 10.1097/00006454-198205000-00005. [DOI] [PubMed] [Google Scholar]
  13. Griffith R. D., Miller O. F., 3rd Erythema multiforme following diphtheria and tetanus toxoid vaccination. J Am Acad Dermatol. 1988 Oct;19(4):758–759. doi: 10.1016/s0190-9622(88)80355-4. [DOI] [PubMed] [Google Scholar]
  14. Howland W. W., Golitz L. E., Weston W. L., Huff J. C. Erythema multiforme: clinical, histopathologic, and immunologic study. J Am Acad Dermatol. 1984 Mar;10(3):438–446. doi: 10.1016/s0190-9622(84)80090-0. [DOI] [PubMed] [Google Scholar]
  15. Huff J. C., Weston W. L. Recurrent erythema multiforme. Medicine (Baltimore) 1989 May;68(3):133–140. doi: 10.1097/00005792-198905000-00001. [DOI] [PubMed] [Google Scholar]
  16. Huff J. C., Weston W. L., Tonnesen M. G. Erythema multiforme: a critical review of characteristics, diagnostic criteria, and causes. J Am Acad Dermatol. 1983 Jun;8(6):763–775. doi: 10.1016/s0190-9622(83)80003-6. [DOI] [PubMed] [Google Scholar]
  17. Hurwitz S. Erythema multiforme: a review of its characteristics, diagnostic criteria, and management. Pediatr Rev. 1990 Jan;11(7):217–222. doi: 10.1542/pir.11-7-217. [DOI] [PubMed] [Google Scholar]
  18. 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):90–93. doi: 10.1007/s004310050561. [DOI] [PubMed] [Google Scholar]
  19. Legrain V., Lejean S., Taïeb A., Guillard J. M., Battin J., Maleville J. Infantile acute hemorrhagic edema of the skin: study of ten cases. J Am Acad Dermatol. 1991 Jan;24(1):17–22. doi: 10.1016/0190-9622(91)70002-j. [DOI] [PubMed] [Google Scholar]
  20. Legrain V., Taïeb A., Sage T., Maleville J. Urticaria in infants: a study of forty patients. Pediatr Dermatol. 1990 Jun;7(2):101–107. doi: 10.1111/j.1525-1470.1990.tb00663.x. [DOI] [PubMed] [Google Scholar]
  21. Maleville J., Massicot P., Ponge A., Guillard J. M., Sarrat P., Guillet G. Aspects clinique et étiologique de l'érythème polymorphe. A propos de quarante observations. Sem Hop. 1983 Mar 10;59(10):671–675. [PubMed] [Google Scholar]
  22. Mortureux P., Léauté-Labrèze C., Legrain-Lifermann V., Lamireau T., Sarlangue J., Taïeb A. Acute urticaria in infancy and early childhood: a prospective study. Arch Dermatol. 1998 Mar;134(3):319–323. doi: 10.1001/archderm.134.3.319. [DOI] [PubMed] [Google Scholar]
  23. Prendiville J. S., Hebert A. A., Greenwald M. J., Esterly N. B. Management of Stevens-Johnson syndrome and toxic epidermal necrolysis in children. J Pediatr. 1989 Dec;115(6):881–887. doi: 10.1016/s0022-3476(89)80736-x. [DOI] [PubMed] [Google Scholar]
  24. Rasmussen J. E. Erythema multiforme in children. Response to treatment with systemic corticosteroids. Br J Dermatol. 1976 Aug;95(2):181–186. doi: 10.1111/j.1365-2133.1976.tb00824.x. [DOI] [PubMed] [Google Scholar]
  25. Renfro L., Grant-Kels J. M., Feder H. M., Jr, Daman L. A. Controversy: are systemic steroids indicated in the treatment of erythema multiforme? Pediatr Dermatol. 1989 Mar;6(1):43–50. doi: 10.1111/j.1525-1470.1989.tb00266.x. [DOI] [PubMed] [Google Scholar]
  26. Roujeau J. C., Beani J. C., Dubertret L., Guillaume J. C., Jeanmougin M., Danan G., Lagier G., Benichou C. Photosensibilité cutanée médicamenteuse. Résultats d'une Réunion de Consensus. Therapie. 1989 May-Jun;44(3):223–227. [PubMed] [Google Scholar]
  27. Roujeau J. C. What is going on in erythema multiforme? Dermatology. 1994;188(4):249–250. doi: 10.1159/000247159. [DOI] [PubMed] [Google Scholar]
  28. Salomon D., Saurat J. H. Erythema multiforme major in a 2-month-old child with human immunodeficiency virus (HIV) infection. Br J Dermatol. 1990 Dec;123(6):797–800. doi: 10.1111/j.1365-2133.1990.tb04200.x. [DOI] [PubMed] [Google Scholar]
  29. Schofield J. K., Tatnall F. M., Leigh I. M. Recurrent erythema multiforme: clinical features and treatment in a large series of patients. Br J Dermatol. 1993 May;128(5):542–545. doi: 10.1111/j.1365-2133.1993.tb00232.x. [DOI] [PubMed] [Google Scholar]
  30. Sontheimer R. D., Garibaldi R. A., Krueger G. G. Stevens-Johnson syndrome associated with Mycoplasma pneumoniae infections. Arch Dermatol. 1978 Feb;114(2):241–244. [PubMed] [Google Scholar]
  31. Tay Y. K., Huff J. C., Weston W. L. Mycoplasma pneumoniae infection is associated with Stevens-Johnson syndrome, not erythema multiforme (von Hebra). J Am Acad Dermatol. 1996 Nov;35(5 Pt 1):757–760. doi: 10.1016/s0190-9622(96)90732-x. [DOI] [PubMed] [Google Scholar]
  32. Teillac D., Marsol P., Richard P., Hubert P., Roujeau J. C., Cloup M., De Frost Y. Nécrolyse épidermique toxique de l'enfant (syndrome de Lyell). A propos de 18 cas. Arch Fr Pediatr. 1987 Oct;44(8):583–587. [PubMed] [Google Scholar]
  33. Weston W. L., Morelli J. G., Rogers M. Target lesions on the lips: childhood herpes simplex associated with erythema multiforme mimics Stevens-Johnson syndrome. J Am Acad Dermatol. 1997 Nov;37(5 Pt 2):848–850. doi: 10.1016/s0190-9622(97)80009-6. [DOI] [PubMed] [Google Scholar]
  34. Wolf P., Soyer H. P., Fink-Puches R., Huff J. C., Kerl H. Recurrent post-herpetic erythema multiforme mimicking polymorphic light and juvenile spring eruption: report of two cases in young boys. Br J Dermatol. 1994 Sep;131(3):364–367. doi: 10.1111/j.1365-2133.1994.tb08525.x. [DOI] [PubMed] [Google Scholar]
  35. Wolkenstein P., Latarjet J., Roujeau J. C., Duguet C., Boudeau S., Vaillant L., Maignan M., Schuhmacher M. H., Milpied B., Pilorget A. Randomised comparison of thalidomide versus placebo in toxic epidermal necrolysis. Lancet. 1998 Nov 14;352(9140):1586–1589. doi: 10.1016/S0140-6736(98)02197-7. [DOI] [PubMed] [Google Scholar]

Articles from Archives of Disease in Childhood are provided here courtesy of BMJ Publishing Group

RESOURCES