Diagnosis and management of Stevens-Johnson syndrome/toxic epidermal necrolysis

Clin Dermatol. 2020 Nov-Dec;38(6):607-612. doi: 10.1016/j.clindermatol.2020.06.016. Epub 2020 Jun 30.


Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) is a rare, immunologically mediated cutaneous adverse reaction characterized by mucous membrane and epidermal detachment, with a mortality ranging from 15% to 25%. Risk factors for the development of SJS/TEN include immune dysregulation, active malignancy, and genetic predisposition. Medications are the most common cause, particularly antimicrobials, antiepileptics, allopurinol, and nonsteroidal anti-inflammatory medications. Drug-specific CD8 T-cells and natural killer cells are thought to be the major inducers of keratinocyte apoptosis via release of soluble cytotoxic mediators, including Fas ligand, perforin/granzyme, tumor necrosis factor, and granulysin. When SJS/TEN is suspected clinically, appropriate therapy should be instituted without delay. All patients should be managed initially in an intensive care unit or burn unit under a multidisciplinary team of physicians experienced in the care of patients with SJS/TEN. Available data support the use of various pharmacologic agents to halt disease progression and improve outcomes, but no single drug has been found to be superior or beneficial for all patients. Future research should focus on developing a better understanding of the genetic susceptibility and immunopathophysiology of the disease, as well as novel diagnostic and therapeutic targets to improve patient outcomes.

Publication types

  • Review

MeSH terms

  • Anti-Infective Agents / adverse effects
  • Anti-Inflammatory Agents, Non-Steroidal / adverse effects
  • Anticonvulsants / adverse effects
  • Antigens, Differentiation, T-Lymphocyte / metabolism
  • Apoptosis / immunology
  • CD8-Positive T-Lymphocytes / immunology
  • Fas Ligand Protein / metabolism
  • Genetic Predisposition to Disease
  • Humans
  • Immune System Diseases / complications
  • Keratinocytes / immunology
  • Killer Cells, Natural / immunology
  • Neoplasms / complications
  • Perforin / metabolism
  • Risk Factors
  • Stevens-Johnson Syndrome / diagnosis*
  • Stevens-Johnson Syndrome / genetics
  • Stevens-Johnson Syndrome / immunology*
  • Stevens-Johnson Syndrome / therapy
  • Tumor Necrosis Factor-alpha / metabolism


  • Anti-Infective Agents
  • Anti-Inflammatory Agents, Non-Steroidal
  • Anticonvulsants
  • Antigens, Differentiation, T-Lymphocyte
  • Fas Ligand Protein
  • GNLY protein, human
  • Tumor Necrosis Factor-alpha
  • Perforin