title: Treatment of toxic epidermal necrolysis with high-dose intravenous immunoglobulins: multicenter retrospective analysis of 48 consecutive cases.
abstract: OBJECTIVE
  To evaluate the effect of high-dose intravenous immunoglobulin (IVIG) in toxic epidermal necrolysis (TEN), parameters that may affect response to treatment, and the effect of different IVIG batches on Fas-mediated cell death.
  
  
  METHODS
  Multicenter retrospective analysis of 48 consecutive TEN patients treated with IVIG.
  
  
  METHODS
  Fourteen university hospital dermatology centers in Europe and the United States.
  
  
  METHODS
  Forty-eight patients with TEN (skin detachment >10% of their body surface [mean, 44.8%; range, 10%-95%]).
  
  
  METHODS
  Infusion of IVIG in all patients (range, 0.8-5.8 g/kg), and analysis of the ability of different IVIG batches to inhibit Fas-mediated cell death.
  
  
  METHODS
  Objective response to IVIG treatment, final outcome at day 45, parameters that may affect response to IVIG treatment, and tolerance.
  
  
  RESULTS
  Infusion of IVIG (mean total dose, 2.7 g/kg [range, 0.65-5.8 g/kg]; mean consecutive days, 4 [range, 1-5 days]) was associated with a rapid cessation (mean, 2.3 days [range, 1-6 days]) of skin and mucosal detachment in 43 patients (90%) and survival in 42 (88%). Patients who responded to IVIG had received treatment earlier in the course of disease and, on average, higher doses of IVIG. Furthermore, analysis of 35 IVIG batches revealed significant batch-to-batch variations in the capacity of IVIG to inhibit Fas-mediated cell death in vitro.
  
  
  CONCLUSIONS
  Early infusion of high-dose IVIG is safe, well tolerated, and likely to be effective in improving the survival of patients with TEN. We recommend early treatment with IVIG at a total dose of 3 g/kg over 3 consecutive days (1 g/kg per day for 3 days).
authors.affiliation: Department of Dermatology, Geneva University Medical School, Switzerland.
authors.forename: Christa
authors.initials: C
authors.lastname: Prins
authors.forename: Francisco A
authors.initials: FA
authors.lastname: Kerdel
authors.forename: R Steven
authors.initials: RS
authors.lastname: Padilla
authors.forename: Thomas
authors.initials: T
authors.lastname: Hunziker
authors.forename: Sergio
authors.initials: S
authors.lastname: Chimenti
authors.forename: Isabelle
authors.initials: I
authors.lastname: Viard
authors.forename: Davide N
authors.initials: DN
authors.lastname: Mauri
authors.forename: Kirsten
authors.initials: K
authors.lastname: Flynn
authors.forename: Jennifer
authors.initials: J
authors.lastname: Trent
authors.forename: David J
authors.initials: DJ
authors.lastname: Margolis
authors.forename: Jean-Hilaire
authors.initials: JH
authors.lastname: Saurat
authors.forename: Lars E
authors.initials: LE
authors.lastname: French
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D017209:Apoptosis; D002648:Child; D002675:Child, Preschool; D053222:Fas Ligand Protein; D005260:Female; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000008:administration & dosage* / Q000494:pharmacology; D066298:In Vitro Techniques; D015603:Keratinocytes / Q000502:physiology; D008297:Male; D008562:Membrane Glycoproteins / Q000037:antagonists & inhibitors / Q000502:physiology; D008875:Middle Aged; D012189:Retrospective Studies; D013262:Stevens-Johnson Syndrome / Q000188:drug therapy* / Q000503:physiopathology
chemical_list: C505661:FASLG protein, human; D053222:Fas Ligand Protein; D016756:Immunoglobulins, Intravenous; D008562:Membrane Glycoproteins