title: Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: An Analysis of Triggers and Implications for Improving Prevention.
abstract: BACKGROUND
  Stevens-Johnson syndrome and toxic epidermal necrolysis are severe mucocutaneous adverse drug reactions characterized by extensive epidermal detachment. The mortality rates have been reported to vary between 1% and 5% for Stevens-Johnson syndrome and 25% and 35% for patients with toxic epidermal necrolysis. Studies have shown that early recognition and prompt withdrawal of the causative agent leads to increased patient survival.
  
  
  METHODS
  A retrospective chart review was conducted on 64 patients admitted to Vancouver General Hospital with a diagnosis of Stevens-Johnson syndrome or toxic epidermal necrolysis from 2001 to 2011. The aim of this study was to identify the medications most often implicated in triggering Stevens-Johnson syndrome and toxic epidermal necrolysis, as well as to delineate the timeline of identification and removal of these triggers.
  
  
  RESULTS
  A trigger was identified in 75% of cases. Allopurinol was the single most common offending agent (20% of cases). Anticonvulsants and antibiotics were common triggers. The offending agent was often removed at time of hospital admission/diagnosis but not at onset of symptoms. A history of prior culprit drug exposure with previous mucocutaneous adverse reaction was noted in 19% of cases with identified triggers. Asians and Native North Americans had a higher mortality than whites, and Asians more frequently had allopurinol as a trigger.
  
  
  CONCLUSIONS
  The onset and high mortality rate of Stevens-Johnson syndrome/toxic epidermal necrolysis may be related to unawareness of the early signs and symptoms of Stevens-Johnson syndrome and toxic epidermal necrolysis, the common drug triggers that cause it, and what investigations (human leukocyte antigen typing in Asians) can be done to prevent it.
authors.affiliation: Department of Dermatology and Skin Science, University of British Columbia, Vancouver, Canada.
authors.forename: Monica A
authors.initials: MA
authors.lastname: Miliszewski
authors.affiliation: Department of Dermatology and Skin Science, University of British Columbia, Vancouver, Canada; Division of Dermatology, Department of Medicine, Queen's University, Kingston, Ont, Canada.
authors.forename: Mark G
authors.initials: MG
authors.lastname: Kirchhof
authors.affiliation: Division of Plastic Surgery, University of British Columbia, Vancouver, Canada.
authors.forename: Sheena
authors.initials: S
authors.lastname: Sikora
authors.affiliation: Division of Plastic Surgery, University of British Columbia, Vancouver, Canada.
authors.forename: Anthony
authors.initials: A
authors.lastname: Papp
authors.affiliation: Department of Dermatology and Skin Science, University of British Columbia, Vancouver, Canada; Child and Family Research Institute, University of British Columbia, Vancouver, Canada. Electronic address: dutz@interchange.ubc.ca.
authors.forename: Jan P
authors.initials: JP
authors.lastname: Dutz
mesh_terms: D000328:Adult; D000368:Aged; D000493:Allopurinol / Q000009:adverse effects*; D000900:Anti-Bacterial Agents / Q000009:adverse effects*; D000927:Anticonvulsants / Q000009:adverse effects*; D044466:Asian People / Q000706:statistics & numerical data; D001955:British Columbia; D005260:Female; D006074:Gout Suppressants / Q000009:adverse effects*; D006680:HLA Antigens / Q000235:genetics; D006801:Humans; D007198:Indians, North American / Q000706:statistics & numerical data; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D013262:Stevens-Johnson Syndrome / Q000209:etiology* / Q000235:genetics / Q000401:mortality / Q000517:prevention & control; D044465:White People / Q000706:statistics & numerical data
chemical_list: D000900:Anti-Bacterial Agents; D000927:Anticonvulsants; D006074:Gout Suppressants; D006680:HLA Antigens; D000493:Allopurinol
keywords: Allopurinol; Drug reactions; HLA testing; Phenytoin; Stevens-Johnson syndrome; Sulfamethoxazole-trimethoprim; Toxic epidermal necrolysis