A 10-year retrospective cohort study of the management of toxic epidermal necrolysis and Stevens-Johnson syndrome in a New South Wales state referral hospital from 2006 to 2016.

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Toxic epidermal necrolysis (TEN) and Stevens-Johnson syndrome (SJS) are life-threatening severe cutaneous drug reactions. There have been no Australian data published since 2007. To explore whether there is an association between prognosis and the type of systemic immune-modulation treatment administered in a cohort of patients with SJS, SJS-TEN overlap, and TEN at the New South Wales State SJS/TEN referral center - Concord Repatriation General Hospital. This is a retrospective, single center, cohort study of patients admitted with SJS/TEN from January 1, 2006, to December 31, 2016, at Concord Repatriation General Hospital. Data on demographic information, the causative agent, treatment, and final survival outcome were analyzed. Forty-two patients included: 26 (62%) with TEN, six (14%) with SJS/TEN overlap, and 10 (24%) with SJS. Overall mortality was 19% (n=8), and seven suffered TEN. The average age of those who died was 60years. Eighty-one percent of patients were managed within the burn unit. Twenty-nine patients (70%) received IVIG within this group; 13 individuals also received systemic corticosteroids. Seven (17%) were managed with corticosteroid therapy alone. The incidence of death was 0% in the combined IVIG and corticosteroid group. This series of 42 patients contributes valuable information to a serious condition with low global incidence and high mortality. There appears to be an apparent reduced mortality in the group of SJS/TEN patients managed with combined IVIG and corticosteroid. Larger cohorts are required to validate this relationship due to the risk of bias inherent to the retrospective study design and small sample size.