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 EuroSCARâ€™s IVIG groups contained a higher proportion of TEN patients than did their corticosteroid-only or supportive careâ€“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.
This study was approved by the Institutional Review Board of the University of Kansas Hospital, Kansas City, KS. Of 14