ACUTE RESPIRATORY FAILURE AS AN INITIAL SIGN OF ZONISAMIDE-INDUCED STEVENS-JOHNSON SYNDROME

Abstract

SESSION TITLE: Tuesday Medical Student/Resident Case Report Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/22/2019 01:00 PM - 02:00 PM INTRODUCTION: Stevens-Johnson Syndrome (SJS) and toxic epidermal necrosis (TEN) are severe drug cutaneous reaction. The offenders include zonisamide, a sulfonamide antieptileptic [1]. Acute respiratory failure is not uncommon in SJS/TEN -- about 25% of patients require mechanical ventilation, which has been associated with poor prognosis [2]. Zonisamide-induced SJS with early acute respiratory failure has not been reported previously. CASE PRESENTATION: A 30 year-old female with no previous allergy, cancer, or lung disease, presented with a one-day history of fever 38.9 °C, oral pain, and rash. She was started on zonisamide for epilepsy 21 days prior. She was on no other medication and denied cough, chest pain, or dyspnea. On admission, pulse oximetry (SpO2) was 94%. The diagnosis of SJS was confirmed by diffuse skin erythema, a blister involving 1% body surface area (BSA), left conjunctiva blister, and oral mucosa sloughing. Within hours, she became lethargic and SpO2 sustained around 85% with 6 liters/min nasal cannula. Chest x-ray was normal. Arterial blood gas while on oxygen showed pH 7.31, PCO2 51 mmHg and PO2 133 mmHg. Nasotracheal tube was placed with fiber-optic guidance. During intubation, no mucosal lesion was observed in the trachea. After intubation, her hypoxia and hypercarbia quickly corrected. All cultures, serology and virology reported negative. She was treated with high dose steroids and given diligent care of the skin, eye, and oral mucosa. Over the next two days, skin blistering progressed to about 20% BSA. Steroids was discontinued; she was given a course of cyclosporine. Her cutaneous and mucosal lesions stabilized and she was successfully extubated after a week. DISCUSSION: So far, there are two case reports in the US and about a dozen worldwide of zonisamide-induced SJS/TEN, but none reported respiratory failure. The etiology of respiratory failure in SJS/TEN includes bronchial epithelial lesions, pneumonia, or ARDS induced by septic shock [2]. Our patient's early-onset respiratory failure was likely from hypoventilation, due to trismus caused by oral ulceration. Nasal intubation effectively bypassed the oropharynx, and was much less invasive than tracheostomy. Fiber-optic guided intubation directly observed the airway mucosa and helped to differentiate the cause of respiratory failure. Our case demonstrated that significant mucosal lesions may proceed cutaneous bullae formation. We concurred with many previous reports that aggressive supportive care remains the mainstay of SJS/TEN treatment, while steroids or other immunosuppressant have limited efficacy [3]. CONCLUSIONS: Respiratory failure is a common complication of SJS and TEN. Early supportive care of the respiratory system, from oxygen supplementation to mechanical ventilation, is a crucial part of the multidisciplinary treatment of SJS/TEN. Nasotracheal tube can establish effective airway in SJS/TEN patients. Reference #1: Vival KL, Mancl K, Seminario-Vidal L. Stevens-Johnson syndrome/toxic epidermal necrosis associated with zonisamide. Clin Case Rep. 2018; 6(2): 258-261. https://doi.org/10.1002/ccr3.1288 Reference #2: de Prost N, Mekontso-Dessap A, Valeyrie-Allanore L, et.al. Acute respiratory failure in patients with toxic epidermal necrosis: clinical features and factors associated with mechanical ventilation. Crit Care Med. 2014; 42(1): 118-28. https://doi.org/10.1097/CCM.0b013e31829eb94f Reference #3: Dodiuk-Gad R, Chung WH, Valeyrie-Allanore L, et.al. Stevens-Johnson Syndrome and toxic epidermal necrosis: an update. Am J Clin Dermatol. 2015; 16: 475-493. https://doi.org/10.1007/s402.57-015-0158-0 DISCLOSURES: No relevant relationships by Bhavinkumar Dalal, source=Web Response No relevant relationships by Yifan Pang, source=Web Response no disclosure on file for Renzhong Ran; No relevant relationships by Stephanee Schrader, source=Web Response