End-Stage Respiratory Failure Secondary to Bronchiolitis Obliterans Syndrome Induced by Toxic Epidermal Necrosis, Also Known as Lyell Syndrome: A Case Report

Transplant Proc. 2021 May;53(4):1371-1374. doi: 10.1016/j.transproceed.2021.03.020. Epub 2021 Apr 23.

Abstract

Background: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare but serious dermatologic diseases. They can be associated with systemic manifestations such as bronchiolitis obliterans syndrome (BOS). SJS/TEN-induced BOS is associated with a poor prognosis, and no guidelines exist regarding its management. Several case reports have described the association between SJS/TEN and BOS, with few patients undergoing lung transplantation as a last resort therapy. Unfortunately, in the published reports, none of the transplanted patients were observed for a long period of time after the transplantation; therefore, the long-term mortality as well as the risk of recurrence of BOS could not be inferred from these reports.

Case report: We present the case of a young patient diagnosed with SJS complicated by BOS and end-stage respiratory failure refractory to corticosteroid therapy. She underwent bilateral lung transplantation with an outstanding outcome at 5-year follow-up.

Conclusion: SJS/TEN-induced BOS might have a favorable evolution and long-term outcomes following lung transplantation. However, prospective studies are needed to confirm this finding.

Publication types

  • Case Reports

MeSH terms

  • Adrenal Cortex Hormones / therapeutic use
  • Bronchiolitis Obliterans / complications*
  • Bronchiolitis Obliterans / etiology
  • Child
  • Epidermis / pathology
  • Female
  • Follow-Up Studies
  • Forced Expiratory Volume
  • Humans
  • Lung Transplantation
  • Respiratory Insufficiency / complications*
  • Respiratory Insufficiency / surgery
  • Stevens-Johnson Syndrome / diagnosis*
  • Stevens-Johnson Syndrome / etiology
  • Vital Capacity

Substances

  • Adrenal Cortex Hormones