Analysis of intravenous immunoglobulin for the treatment of toxic epidermal necrolysis using SCORTEN: The University of Miami Experience.
BACKGROUND
Toxic epidermal necrolysis (TEN) is a rare, life-threatening condition caused by certain medications. Keratinocytes affected by TEN have been found to undergo apoptosis mediated by Fas-FasL interactions. Treatment with intravenous immunoglobulin (IVIG) has been proposed to inhibit this interaction.


OBJECTIVE
To demonstrate the effectiveness of IVIG therapy in reducing mortality in patients with TEN.


METHODS
A retrospective analysis of 16 consecutive patients with TEN who were treated with IVIG. The SCORTEN system, a validated predictor of TEN mortality, was used to analyze the data of these patients. Using SCORTEN, we compared the predicted mortality of our patient population with observed mortality.


METHODS
Dermatology inpatient unit at a university-affiliated hospital.


METHODS
All 16 patients received IVIG treatment daily for 4 days. Fifteen patients received 1 g/kg per day and 1 patient received 0.4 g/kg per day.


METHODS
For each patient, causes of TEN and other medical problems were documented prior to IVIG therapy, as were the 7 independent SCORTEN risk factors.


RESULTS
One patient died. Based on the SCORTEN system, 5.81 patients were expected to die. These mortality rates were compared using the standardized mortality ratio (SMR) analysis ([Sigma observed deaths/Sigma expected deaths] x 100) to determine the efficacy of this treatment, which showed that patients with TEN treated with IVIG were 83% less likely to die than those not treated with IVIG (SMR = 0.17; 95% confidence interval, 0.0-0.96).


CONCLUSIONS
Based on comparison of our observed mortality rate with the SCORTEN-predicted mortality rate, treatment with IVIG significantly decreased mortality in patients with TEN.
Trent
Jennifer T
JT
Department of Dermatology and Cutaneous Surgery, University of Miami School of Medicine, FL 33136, USA.
Kirsner
Robert S
RS
Romanelli
Paolo
P
Kerdel
Francisco A
FA
D000328:Adult; D005260:Female; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D012720:Severity of Illness Index; D013262:Stevens-Johnson Syndrome / Q000188:drug therapy* / Q000401:mortality / Q000473:pathology; D015996:Survival Rate
D016756:Immunoglobulins, Intravenous