Early Treatment With Nonsucrose Intravenous Immunoglobulin in a Burn Unit Reduces Toxic Epidermal Necrolysis Mortality

June 2013 | Volume 12 | Issue 6 | Original Article | 679 | Copyright © June 2013


Daniel J. Aires MD JD,a Garth Fraga MD,b Richard Korentager MD,c Coleman P. Richie MD,d Smita Aggarwal MD,e Jo Wick PhD,f and Deede Y. Liu MDa

aDivision of Dermatology, Department of Internal Medicine, bDepartment of Pathology and Laboratory Medicine, cDepartment of Plastic Surgery and Burns, fDepartment of Biostatistics, University of Kansas Medical Center, Kansas City, KS dDepartment of Dermatology, Stritch School of Medicine, Loyola University Chicago, Chicago, IL eDepartment of Dermatology, Vanderbilt School of Medicine, Nashville, TN

Abstract
BACKGROUND: Intravenous immunoglobulin (IVIG) can be used to treat potentially deadly toxic epidermal necrolysis (TEN), milder Stevens Johnson Syndrome (SJS) and intermediate TEN/SJS overlap. Some formularies now deny IVIG for TEN based on the EuroSCAR TEN/SJS study that reported a nonsignificant trend toward increased mortality in 75 IVIG-treated TEN/SJS patients; of note the IVIG patients had more TEN and less SJS than patients in other treatment arms. EuroSCAR data on mortality among the 25 IVIG-treated TEN patients, use of nonsucrose IVIG, and admission to specialized settings such as burn units was not disclosed. The impact of treatment setting (specialized unit vs general ward) on IVIG efficacy has not previously been studied.
OBJECTIVE: To evaluate efficacy of treating TEN with early nonsucrose IVIG in a burn unit.
METHODS: Data were retrospectively collected from 13 IVIG-treated TEN patients admitted to a burn unit over a 6-year period.
RESULTS: We report 0% mortality among 13 IVIG-treated TEN patients. Mortality was significantly lower than predicted by SCORTEN. Mortality was also significantly lower than the EuroSCAR groups receiving IVIG (P<.005), supportive care (P<.018), and corticosteroids only (P<.046).
CONCLUSION: TEN patients may benefit from early nonsucrose IVIG administered in burn units or other specialized settings.

J Drugs Dermatol. 2013;12(6):679-684.

INTRODUCTION

Toxic epidermal necrolysis (TEN) is a rare, life-threatening blistering skin condition that is generally caused by exposure to certain high-risk medications.1 Patients typically present with fever, sore throat, and sore eyes, and subsequently develop widespread apoptotic mucocutaneous lesions that progress to full thickness epidermal necrosis.2 TEN involves greater than 30% total body surface area (TBSA). Stevens-Johnson Syndrome (SJS) is generally thought to be a milder form of TEN with TBSA involvement of less than 10% and significantly lower mortality. SJS-TEN overlap is an intermediate condition with TBSA of 10% to 30%. TEN mortality rates can be >30%, whereas SJS is less deadly with a mortality rate of 5%, and SJS-TEN overlap falls somewhere in between.3 High risk of mortality makes prompt diagnosis and treatment of TEN imperative.
By the 1990s, it was known that the cellular death receptor FAS (CD95) plays a role in TEN and SJS, and IVIG inhibits CD95 ligation, so physicians started treating TEN and SJS with IVIG.4,5 Subsequent research indicated that IVIG may act via the immune molecule granulysin.6 Recently, there has been controversy about the use of IVIG in TEN. Two studies showed benefit with IVIG,8,9 whereas 3 showed no benefit, and one, the 2008 EuroSCAR study, showed a nonsignificant trend toward harm.10-12 EuroSCAR found that in a mixed TEN/overlap/SJS group assembled from multiple hospitals in different countries there was a nonsignificant trend toward higher mortality with IVIG.7 EuroSCAR’s IVIG groups contained a higher proportion of TEN patients than did their corticosteroid-only or supportive care–only groups. EuroSCAR included 10 TEN patients treated with IVIG and corticosteroids and 15 TEN patients treated with IVIG monotherapy, but mortality for these TEN-only groups was not separately disclosed, nor was the percent that received nonsucrose IVIG or were admitted to burn units or similar specialized settings.
The present study reports IVIG monotherapy treatment of 13 TEN patients admitted to the burn unit at an academic teaching hospital and treated upon admission with with nonsucrose IVIG. Outcomes from the present study are in line with those previously reported for IVIG-treated TEN in a specialized dermatology unit8 and are significantly better than those reported in EuroSCAR.

METHODS

This study was approved by the Institutional Review Board of the University of Kansas Hospital, Kansas City, KS. Of 14
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