Toxic epidermal necrolysis in adult patients: Experience from the West Australian Collaboration

Toxic epidermal necrolysis (TEN) is a rare and life-threatening mucocutaneous disease triggered by a reaction to a drug. Despite reported mortality of 30%, management differs between healthcare settings. Our hospital was established in February 2015 becoming the new state burns centre in Western Australia (WA). Following this, we collaborated on comprehensive multidisciplinary guidelines for the management of TEN. These guidelines are updated annually to reflect the weight of emerging evidence in managing TEN. Our aim was to review the management and outcomes of TEN patients presenting to our hospital between February 2015 and May 2021 (inclusive). We collected data for 10 patients on year, age, ethnicity, gender, medical history, culprit drug and exposure, SCORTEN, length of stay, maximum percentage of skin detachment, mucosal surface involvement, ophthalmic amniotic membrane transplant, burns unit input/admission, intensive care unit admission, weight, systemic treatment(s), complications and outcome. We excluded 7 out of 17 flagged patients who did not strictly meet the definition of TEN as greater than 30% epidermal detachment, with epidermal detachment defined as bullae, erosions, and/or positive Nikolsky. We found that the mortality rate in WA from TEN is improving compared with two previous WA studies, with a mortality rate in our study of 20% (2 deaths). Though limited by small sample size and retrospective design, our study suggests a shift towards at least one systemic therapy per patient (most commonly cyclosporine), the growing use of etanercept and the ophthalmic use of amniotic membrane transplants. It demonstrates the importance of burns unit input and the utility of comprehensive multidisciplinary guidelines. While the management and outcomes of TEN patients in WA are continuing to improve, we support calls for large registry data to facilitate evidence growth and collaboration for this rare life-threatening condition.