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Toxic epidermal necrolysis treatment and outcomes: a retrospective analysis of 10 patients treated with intravenous immunoglobulin

intravenous immunoglobulin Toxic epidermal necrolysis (TEN) and Stevens–Johnson syndrome (SJS) are acute, life-threatening conditions characterized by skin necrosis with exfoliation of the epidermis and mucous membranes. SJS, SJS-TEN overlap (SJS-TEN), and TEN are considered on the same spectrum, with SJS defined as skin exfoliation <10% body surface area (BSA), SJS-TEN overlap 10–30%, and TEN as >30% BSA. SJS-TEN is a rare condition with a combined incidence of 2–7 cases per million. Mortality rates range from 1 to 3% for SJS and 30 to 50% for TEN. Treatment for SJS, SJS/TEN, and TEN has been studied more extensively over the past few decades. Multiple studies have been performed to evaluate treatment because mortality remains high. Various treatment strategies have been used over the years, but there has been no consensus on optimal treatment. As SJS and TEN are often precipitated by a medication, treatment usually begins with immediate cessation of the offending drug. Expedited transfer of the patient to a burn unit for supportive care may also significantly improve survival. However, even in the care of an experienced burn unit, the mortality for SJS-TEN remains high, leading investigators to evaluate additional treatments. Systemic corticosteroids, intravenous immunoglobulin (IVIG), or a combination of the two have been the most studied treatments over the years with various results. Systemic steroids were the initial mainstay of treatment, but some studies have shown no benefit or increased mortality when used alone. Other studies have shown increased survival in corticosteroid treatment groups. Later studies evaluated treatment efficacy with IVIG. A study by Jagadeesan et al. showed increased survival using steroids with low-dose IVIG, when compared with steroids alone. Yang et al. also compared a combination of IVIG–steroids to steroids alone. Results showed a trend towards decreased mortality, but the result was not statistically significant. Several other clinical studies using IVIG as an adjuvant treatment in patients with TEN have shown increased survival. However, there have also been multiple studies showing no survival benefit with IVIG. Given this, there is currently no consensus on a role for adjuvant therapy, either corticosteroids or IVIG, in the treatment of SJS-TEN. With our study, we aim to analyze whether IVIG affects the mortality rate in patients with TEN or the SJSTEN overlap. This information may provide further information about the optimal treatment for patients with these life-threatening skin diseases. Patients with the SJS-TEN overlap and TEN were included in this study. The study protocol qualified for IRB exemption. A coding search was performed for all ICD-9 diagnoses of SJS, SJS/TEN overlap syndrome, or TEN at our practice during the past 10 years. The relevant patient charts were then reviewed to gather relevant data. Patients treated with IVIG alone that had 10% or greater BSA involvement were included in the study for analysis. Patients with SJS were excluded from the study because baseline mortality is less than 2%. In addition, all the SJS cases were treated conservatively. Diagnosis was based on clinical findings by the dermatology team or the burn unit physicians that admitted the patient. Skin biopsy analysis was performed to confirm many of the diagnoses (Fig. 1). A total of 10 patients with SJS-TEN or TEN were included in the study. All of the patients were admitted to either the Detroit Receiving Hospital Burn Unit or affiliated hospitals of the Detroit Medical Center. The patients were managed per burn unit protocols with non-adherent dressings, fluid– electrolyte management, pain control, antacids, and oral hygiene. Ophthalmic and systemic antibiotics were administered as needed. In certain cases, ventilatory sup-