title: Nine years of a single referral center management of Stevens-Johnson syndrome and toxic epidermal necrolysis (Lyell's syndrome).
abstract: BACKGROUND
  Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) corresponds to a rare and acute life-threatening mucocutaneous reactions characterized by extensive necrosis and epidermal detachment. There are no efficacious pharmaceutical interventions proven through large clinical trials.
  
  
  OBJECTIVE
  We sought to study clinical cases admitted in our institution in order to determine which drugs and medical comorbidities or treatments impacted the mortality.
  
  
  METHODS
  In a retrospective study over 9 years we evaluated all patients presenting biopsy-proven SJS or TEN for age, gender, total body surface area involved, causing agents, SCORTEN score, blood transfusion, steroid administration, intubation, length of intensive care stay and death rate. Statistical analysis was done using SPSS statistical software.
  
  
  RESULTS
  The highest incidence of SJS and TEN was in age group of 71-80 years. Of the 30 patients, 30% died from SJS/TEN, mainly due sepsis. For each subgroup SJS/TEN overlap had the highest mortality. The highest mortality was from antibiotic treatment as causing agent. Step-wise regression analysis identified mechanical ventilation requirement and age over 65 years as mortality high-risk factors.
  
  
  CONCLUSIONS
  The most crucial interventions are discontinuation of the offending drug and prompt referral to a burn unit, which helps in early diagnosis and decrease mortality in these diseases.
  
  
  CONCLUSIONS
  When SJS/TEN is caused by antibiotics suspicion of developing fatal sepsis should be high, independently of patients' medical condition.
authors.affiliation: a Centro Hospitalar de Sao Joao , Porto , Portugal.
authors.forename: Diana
authors.initials: D
authors.lastname: Monteiro
authors.affiliation: a Centro Hospitalar de Sao Joao , Porto , Portugal.
authors.forename: Paula
authors.initials: P
authors.lastname: Egipto
authors.affiliation: a Centro Hospitalar de Sao Joao , Porto , Portugal.
authors.forename: Julia
authors.initials: J
authors.lastname: Barbosa
authors.affiliation: a Centro Hospitalar de Sao Joao , Porto , Portugal.
authors.forename: Ricardo
authors.initials: R
authors.lastname: Horta
authors.affiliation: a Centro Hospitalar de Sao Joao , Porto , Portugal.
authors.forename: Jose
authors.initials: J
authors.lastname: Amarante
authors.affiliation: a Centro Hospitalar de Sao Joao , Porto , Portugal.
authors.forename: Pedro
authors.initials: P
authors.lastname: Silva
authors.affiliation: a Centro Hospitalar de Sao Joao , Porto , Portugal.
authors.forename: Alvaro
authors.initials: A
authors.lastname: Silva
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000368:Aged; D000369:Aged, 80 and over; D000900:Anti-Bacterial Agents / Q000009:adverse effects / Q000627:therapeutic use; D001803:Blood Transfusion; D001830:Body Surface Area; D002053:Burn Units; D002648:Child; D002675:Child, Preschool; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D015994:Incidence; D008297:Male; D008875:Middle Aged; D012017:Referral and Consultation; D012121:Respiration, Artificial; D012189:Retrospective Studies; D012307:Risk Factors; D018805:Sepsis / Q000188:drug therapy / Q000209:etiology; D012720:Severity of Illness Index; D013262:Stevens-Johnson Syndrome / Q000209:etiology / Q000473:pathology* / Q000628:therapy*; D028761:Withholding Treatment; D055815:Young Adult
chemical_list: D000900:Anti-Bacterial Agents; D005938:Glucocorticoids
keywords: Lyell’s disease; SCORTEN; Stevens–Johnson’s syndrome; burn unity; toxic epidermal necrolysis