ACUTE GENERALIZED EXENTHEMATOUS PUSTULOSIS MIMICKING SEPTIC SHOCK: UNUSUAL PRESENTATION OF AN UNUSUAL DISEASE

Abstract

SESSION TITLE: Medical Student/Resident Critical Care Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: Acute generalized exanthematous pustulosis (AGEP) is a rare disorder, characterized by the development of acute sterile pustules. It is mostly caused by drugs, with antibiotics being the most likely culprits. AGEP is a self-limiting disease with favorable prognosis in the vast majority of patients. We present a case of a woman, admitted to the intensive care unit (ICU) with an unusually severe form of AGEP mimicking septic shock. CASE PRESENTATION: A 45-year-old female with systemic lupus erythematosus (SLE), underwent left hip replacement due to avascular necrosis. She received prophylactic cephazolin and discharged two days after surgery with an uncomplicated post-operative course. Two days later, she was admitted to the ICU with fever (103.6 F), tachycardia, and hypotension (54/36 mmHg). On physical exam, she had diffuse small pustules on an erythematous base (Fig 1). The incision site showed no signs of infection (Fig. 2). Labs were significant for leukocytosis (WBC 22,000), neutrophilia (18,500), left shift (Bands 13.9), eosinophilia (160), acute kidney injury (Cr 5.3, baseline 0.8), increased inflammatory markers (ESR 19, CRP 32.7), procalcitonin (2.49) and lactic acidosis. Random cortisol was normal and urine analysis was clear. Chest X-Ray showed no infiltrates and left hip CT scan showed no hematoma or abscess. She was initially managed as septic shock, started on broad spectrum antibiotics and required 2 vasopressors. Her hemodynamic status dramatically improved by the second day. Given her typical eruption and no source of infection identified, she was diagnosed with AGEP and antibiotics were discontinued on day 2. Patient continued to improve; vasopressors were weaned off by the third day. Cultures showed no growth. Kidney function and lactate levels normalized by the fifth day and she was discharged from the hospital on day 11. The skin pustules gradually coalesced into plaques (Fig. 3, 4) with subsequent sloughing. DISCUSSION: AGEP is a rare acute cutaneous reaction, with characteristic numerous, pinhead-sized pustules on an edematous erythematous background. It commonly presents with fever, neutrophilia, and skin eruption. Thus, often misinterpreted as an acute infectious process. AGEP is self-limiting, tends to resolve spontaneously with cessation of the offending agent, and no further measures are usually required. Multi-organ involvement is rare and highly atypical of AGEP. In contrast to most reported cases of AGEP, our case demonstrated an atypical presentation with severe systemic involvement and multi-organ failure mimicking septic shock. The patient had SLE and was on chronic corticosteroid treatment, which might explain her unusual life-threatening course. CONCLUSIONS: Prompt diagnosis of AGEP is crucial as the mainstay of treatment is the withdrawal of the offending drug. Every effort should be made to distinguish it from other causes of shock, mainly infection. Reference #1: idoroff A, Halevy S, Bavinck JN, Vaillant L, Roujeau JC. Acute generalized exanthematous pustulosis (AGEP)--a clinical reaction pattern. J Cutan Pathol. 2001;28(3):113-9 Reference #2: Roujeau JC, Bioulac-Sage P, Bourseau C, Guillaume JC, Bernard P, Lok C, et al. Acute generalized exanthematous pustulosis. Analysis of 63 cases. Arch Dermatol. 1991;127(9):1333-8 Reference #3: Krishna S, Ortega-Loayza A, Malakouti N, Brinster N. A rapidly progressive and fatal case of atypical acute generalized exanthematous pustulosis. J Am Acad Dermatol. 2014;71(3):e89-90 DISCLOSURES: No relevant relationships by Mohamed Elmassry, source=Web Response No relevant relationships by Amr Ismail, source=Web Response No relevant relationships by Haneen Mallah, source=Web Response No relevant relationships by Barbara Mantilla, source=Web Response No relevant relationships by Jasmine Sekhon, source=Web Response No relevant relationships by Victor Test, source=Web Response No relevant relationships by Myrian Vinan Vega, source=Web Response