Treatment of toxic epidermal necrolysis with high-dose intravenous immunoglobulins: multicenter retrospective analysis of 48 consecutive cases.
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).
Prins
Christa
C
Department of Dermatology, Geneva University Medical School, Switzerland.
Kerdel
Francisco A
FA
Padilla
R Steven
RS
Hunziker
Thomas
T
Chimenti
Sergio
S
Viard
Isabelle
I
Mauri
Davide N
DN
Flynn
Kirsten
K
Trent
Jennifer
J
Margolis
David J
DJ
Saurat
Jean-Hilaire
JH
French
Lars E
LE
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
C505661:FASLG protein, human; D053222:Fas Ligand Protein; D016756:Immunoglobulins, Intravenous; D008562:Membrane Glycoproteins