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Retrospective evaluation of a TEN/SJS series managed with a new treatment protocol

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journal contribution
submitted on 2024-08-05, 11:51 and posted on 2024-08-05, 11:52 authored by Martin Steinhoff, Joerg Buddenkotte, Wadha Al‐Shafi, Hissa Al‐Marri, Fatima Emam, Mariam Iqneibi, Tim Richard Edmund Harris, Stephen H. Thomas, Syed Muhammad Asad, Hanan Al‐Maslamani, Febu Elizabeth Joy, Lubna Therachiyil, Anh Jochebeth, Rari Leo, Shahad M. Younis, Laith Jamal Abu Raddad, Soha Roger Dargham, Sara Al‐Khawaga

Stevens–Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare, life-threatening diseases without standardized treatment. The mortality rate ranges from 20% to 50%, and can reach up to 90%, based on the severity index SCORTEN. Albeit critical, no active systemic therapeutic regimen with unequivocal benefit exists as of yet; most included cyclosporine A (CyA), immunoglobulins (IVIG) or systemic glucocorticosteroids (GCS). Anti-TNF therapy has been described as beneficial in some reports, and a randomized controlled trial was discontinued prematurely because of death excess in the thalidomide treatment group. In the EuroSCAR cohort study, administration of IVIG resulted in a mortality rate of 34% (IVIG alone) and 18% (IVIG and corticosteroids).

Other Information

Published in: Journal of the European Academy of Dermatology and Venereology
License: http://creativecommons.org/licenses/by-nc-nd/4.0/
See article on publisher's website: https://dx.doi.org/10.1111/jdv.20060

Funding

Hamad Medical Corporation (MRC-01-21-763).

History

Language

  • English

Publisher

Wiley

Publication Year

  • 2024

License statement

This Item is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.

Institution affiliated with

  • Hamad Bin Khalifa University
  • College of Health and Life Sciences - HBKU
  • Hamad Medical Corporation
  • Hamad General Hospital - HMC
  • Dermatology Institute - HMC
  • Qatar University
  • Qatar University Health - QU
  • College of Medicine - QU HEALTH
  • Weill Cornell Medicine - Qatar