Toxic Epidermal Necrolysis vs Lupus Flare?

Abstract

SESSION TITLE: Critical Care 2 SESSION TYPE: Affiliate Case Report Poster PRESENTED ON: Tuesday, October 31, 2017 at 01:30 PM - 02:30 PM INTRODUCTION: Toxic epidermal necrolysis (TEN) and Stevens Johnson syndrome (SJS) are severe mucocutaneous reactions characterized by extensive necrosis and detachment of epidermis. Systemic lupus erythematosus (SLE) patients are at increased risk of SJS/TEN. Here we discuss a patient with SLE who developed TEN. CASE PRESENTATION: A 45 year old female with discoid and systemic lupus erythematosus presented to dermatology with bullous skin lesions 2 days after starting gabapentin, naproxen, and duloxetine. Despite stopping her new medications, she developed a facial rash which spread to her trunk and extremities, affecting 30% body surface area. She had denuded oral mucosa and conjunctival hyperemia with no photosensitivity or eosinophilia. Complements were normal. Wedge biopsy showed acute on chronic vacuolar interface dermatitis with epidermal ulceration and partial to full-thickness epidermal necrosis, suggestive of chronic discoid lupus with superimposed bullous eruption. The patient was admitted to the ICU and treated with intravenous immunoglobulin for three days. Home medications were held. Supportive care included intravenous hydration, reverse isolation in heated environment, specialized wound care, oral and eye care. Methicillin resistant staphylococcus aureus bacteremia was treated. The patient clinically improved and was in reepithelialization phase at time of discharge. DISCUSSION: The incidence of TEN is 0.4-1.2% per million. In patients with SJS/TEN, the prevalence of SLE is estimated to be 1.2% (compared to 0.02-0.05% in the general population). It is unclear if the predisposition for TEN in SLE patients is because of the underlying inflammatory state or because of medications SLE patients often require, such as NSAIDs. Differentiating SLE from TEN is complicated by clinical and histologic similarities. Pathology often demonstrates subepidermal vacuoles, necrotic keratinocytes, and confluent epidermal necrosis, which is found in SLE and TEN. Bullous eruptions and erosive mucosal involvement are typical features in TEN, but can also be found in SLE. This patient developed skin and mucosal lesions after starting new medications and had improvement without steroids, which makes active SLE less likely. Normal complement and serology also supports the clinical diagnosis of TEN over active SLE. CONCLUSIONS: Mortality in patients with SJS and TEN exceeds 10 and 30% respectively. Because the management of active SLE lesions and TEN differ greatly, early recognition of TEN can be life saving. High index of suspicion, early biopsy, and serology can be helpful in differentiating such cases. Reference #1: M. Ziemer, et al, “Stevens-Johnson syndrome and toxic epidermal necrolysis in patients with lupus erythematosus: a descriptive study of 17 cases from a national registry and review of the literature,” Brit J Dermatol 2012; 166 (3), 575-600. Reference #2: Ranario JS, et al. Bullous lesions in a patient with systemic lupus erythematosus. J Clin Aesthet Dermatol 2014; 7:44-49 DISCLOSURE: The following authors have nothing to disclose: Gauri Behari, Sukhdeep Malhi, Bradley Tymchuk, Ronnie Self II, Suman Lata, Ibrahim El-Abbassi No Product/Research Disclosure Information