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Treatment of Epidermal Necrolysis with High-Dose Intravenous Immunoglobulins (IVIg)

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Abstract

High-dose human intravenous immunoglobulins (IVIg) have now been used as a treatment for epidermal necrolysis for several years.

We have reviewed all series involving more than nine patients treated with high-dose IVIg for toxic epidermal necrolysis (TEN) or Stevens-Johnson syndrome (SJS) published in indexed journals. Nine series included a total of 156 patients; among the 156 reported cases, 32 patients died (20.5%). When the analysis was restricted to the five series that included some comparison with expected deaths, the mortality rate observed in patients treated with IVIg was 27% versus an expected rate of 30%. Because of high diversity in study designs and dosages of IVIg used, and because several series included duplicate cases, it was not possible to make more detailed statistical analyses, including individual prognostic factors and IVIg dosages.

In the absence of randomised controlled trials, this review does not provide a definite conclusion on the usefulness of IVIg in SJS or TEN; however, the analysis of published data does not suggest a dramatic efficacy.

We conclude that, in the absence of further studies, IVIg cannot yet be considered the standard of care for SJS or TEN.

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References

  1. Bastuji-Garin S, Rzany B, Stern RS, et al. Clinical classification of cases of toxic epidermal necrolysis, Stevens-Johnson syndrome, and erythema multiforme. Arch Dermatol 1993; 129: 92–6

    Article  PubMed  CAS  Google Scholar 

  2. Shöpf E, Stühmer A, Rzany B, et al. Toxic epidermal necrolysis and Stevens-Johnson syndrome: an epidemiologic study from West Germany. Arch Dermatol 1991; 127: 839–42

    Article  Google Scholar 

  3. Roujeau JC, Stern RS. Severe adverse cutaneous reactions to drugs. N Engl J Med 1994; 331: 1272–85

    Article  PubMed  CAS  Google Scholar 

  4. Bastuji-Garin S, Fouchard N, Bertocchi M, et al. SCORTEN: a severity-of-illness score for toxic epidermal necrolysis. J Invest Dermatol 2000; 115: 149–53

    Article  PubMed  CAS  Google Scholar 

  5. Trent JT, Kirsner RS, Romanelli P, et al. Analysis of Intravenous Immunoglobulin for the treatment of toxic epidermal necrolysis using SCORTEN: the University of Miami experience. Arch Dermatol 2003; 139: 39–43

    Article  PubMed  CAS  Google Scholar 

  6. Campione E, Marulli GC, Carrozo AM, et al. High-dose intravenous immunoglobulin for severe drug reactions: efficacy in toxic epidermal necrolysis. Acta Derm Venereol 2003; 83: 430–2

    PubMed  CAS  Google Scholar 

  7. Brown KM, Silver GM, Halerz M, et al. Toxic epidermal necrolysis: does immunoglobulin make a difference? J Burn Care Rehabil 2004; 25(1): 81–8

    Article  PubMed  CAS  Google Scholar 

  8. Trent JT, Kirsner RS, Romanelli P, et al. Use of SCORTEN to accurately predict mortality in patients with toxic epidermal necrolysis in the United States. Arch Dermatol 2004; 140: 890–2

    Article  PubMed  Google Scholar 

  9. Paul C, Wolkenstein P, Adle H, et al. Apoptosis as a mechanism of keratinocyte death in toxic epidermal necrolysis. Br J Dermatol 1996; 134: 710–4

    Article  PubMed  CAS  Google Scholar 

  10. Nassif A, Bensussan A, Boumsell L, et al. Toxic epidermal necrolysis: effector cells are drug-specific cytotoxic T cells. J Allergy Clin Immunol 2004; 114: 1209–15

    Article  PubMed  CAS  Google Scholar 

  11. Viard I, Wehrli P, Bullani R, et al. Inhibition of toxic epidermal necrolysis by blockage of CD95 with human intravenous immunoglobulin. Science 1998; 282: 490–3

    Article  PubMed  CAS  Google Scholar 

  12. Kazatchkine MD, Kaveri SV. Immunomodulation of autoimmune and inflammatory diseases with intravenous immune globulin. N Engl J Med 2001; 345: 747–55

    Article  PubMed  CAS  Google Scholar 

  13. Stella M, Cassano P, Bolero D, et al. Toxic epidermal necrolysis treated with intravenous high-dose immunoglobulins: our experience. Dermatology 2001; 203: 45–9

    Article  PubMed  CAS  Google Scholar 

  14. Prins C, Kerdel FA, Padilla RS, et al. Treatment of toxic epidermal necrolysis with high-dose intravenous immunoglobulins: multicenter retrospective analysis of 48 consecutive cases. Arch Dermatol 2003; 139: 26–32

    Article  PubMed  CAS  Google Scholar 

  15. Bachot N, Revuz J, Roujeau JC. Intravenous immunoglobulin treatment for Stevens-Johnson syndrome and toxic epidermal necrolysis. Arch Dermatol 2003; 139: 33–6

    Article  PubMed  CAS  Google Scholar 

  16. Prins C, Vittorio C, Padilla RS, et al. Effect of high-dose intravenous immunoglobulin therapy in Stevens-Johnson syndrome: a retrospective, multicenter study. Dermatology 2003; 207: 96–9

    Article  PubMed  CAS  Google Scholar 

  17. Shortt R, Gomez M, Mittman N, et al. Intravenous immunoglobulin does not improve outcome in toxic epidermal necrolysis. J Burn Care Rehabil 2004; 25: 246–55

    Article  PubMed  Google Scholar 

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Acknowledgements

Jean-Claude Roujeau has received research grants from several drug companies for conducting research on severe cutaneous drug reactions. He was also a consultant for LFB, a laboratory marketing immunoglobulins for intravenous administration in France. Dr Faye has no conflicts of interest that are directly relevant to the content of this review.

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Correspondence to Jean-Claude Roujeau.

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Faye, O., Roujeau, JC. Treatment of Epidermal Necrolysis with High-Dose Intravenous Immunoglobulins (IVIg). Drugs 65, 2085–2090 (2005). https://doi.org/10.2165/00003495-200565150-00002

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  • DOI: https://doi.org/10.2165/00003495-200565150-00002

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