Stevens-Johnson syndrome and toxic epidermal necrolysis

Andrea T. Borchers, Jennifer L. Lee, Stanley M Naguwa, Gurtej S. Cheema, M. Eric Gershwin

Research output: Contribution to journalArticlepeer-review

139 Scopus citations

Abstract

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare, but potentially life threatening, diseases characterized by widespread epidermal necrosis, and are predominantly medication-induced. Unfortunately, though they are often associated with long-term debilitating sequelae, there are currently no efficacious pharmaceutical interventions proven through large clinical trials. It has been well established that the epidermal damage in these diseases is due to keratinocyte apoptosis. Although drug-specific T cells are implicated in this process, our understanding of the immunopathology is far from complete. The scenario suggested by today's literature points towards drug-specific CD8+ cytotoxic T cells utilizing perforin/granzyme B trigger keratinocyte apoptosis. Subsequently, there may be an expansion of apoptosis involving the interaction of either membrane-bound or soluble Fas ligand (sFasL) with its receptor Fas. The cellular source of sFasL remains controversial, with both peripheral lymphocytes and keratinocytes themselves as potential candidates. Cytokines produced by T lymphocytes, macrophages or keratinocytes may participate by activating keratinocytes and enhancing their expression of Fas and FasL, or by promoting the skin recruitment of lymphocytes by upregulating adhesion molecules. A better understanding of the underlying immunological mechanisms is required to identify appropriate therapeutic interventions. Finally, clinicians must remain vigilant about drug hypersensitivity to prevent SJS/TEN.

Original languageEnglish (US)
Pages (from-to)598-605
Number of pages8
JournalAutoimmunity Reviews
Volume7
Issue number8
DOIs
StatePublished - Sep 2008

Keywords

  • Apoptosis
  • Fas
  • Perforin/granzyme
  • Stevens-Johnson syndrome
  • Toxic epidermal necrolysis

ASJC Scopus subject areas

  • Immunology
  • Immunology and Allergy

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