FOOLED BY FIBRINOGEN, DISTRACTED BY ESCHERICHIA COLI: AN UNEXPECTED PRESENTATION OF PURPURA FULMINANS

Abstract

SESSION TITLE: Medical Student/Resident Critical Care Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: Purpura fulminans (PF) is a rare, life-threatening dermatologic manifestation of disseminated intravascular coagulation (DIC) most often precipitated by severe sepsis from meningococcemia in children or Group A Streptococcus in adults. It is classically characterized by a classic pattern of cutaneous purpura leading to rapidly progressive skin necrosis and multi-system organ failure due to thrombosis of small and medium-sized blood vessels (1). CASE PRESENTATION: A 28 year old male with acquired immunodeficiency syndrome (AIDS, CD4 count 75), Kaposi’s sarcoma on doxorubicin, and adrenal insufficiency was admitted to the intensive care unit (ICU) for septic shock after being found at home in a pool of liquid stool. He was immediately intubated for profound metabolic acidosis and respiratory failure. The patient was initiated on vasopressors and broad spectrum antibiotics, but developed multi-organ failure requiring continuous renal replacement therapy, ischemic hepatitis, and DIC (Internationalized Normalized Ratio (INR) 4.5, Partial Thromboplastin Time (PTT) 52.8 seconds, D-Dimer 41,857 ng/mL, fibrinogen 280 mg/dL). Blood cultures grew Escherichia coli, likely from gastrointestinal source in the setting of diarrhea. On hospital day two, the patient developed large bullae over a base of well-defined purpuric patches on bilateral lower extremities, scrotum, and left dorsal arm. PF was suspected and definitively diagnosed by dermal biopsy showing scattered late-stage fibrin thrombi in small dermal vascular channels with epidermal and dermal necrosis. The patient survived the ICU admission with gradual resolution of his multi-organ failure but required quadruple amputations due to PF and vasopressor-related digit ischemia. DISCUSSION: Here we describe a case of PF with an unusual causative organism and a non-classic coagulation profile. PF is typically caused by meningococcus, Streptococcus or less commonly Staphylococcus, Vibrio, or Varicella through hypothesized thrombotic pathways involving over activation of tissue factor and relative depletion of protein C and S (1,2). Escherichia coli associated PF is exceptionally rare with only three reported cases (1,3-4). An additional unusual feature of this case was that the patient maintained persistently normal levels of fibrinogen despite the development of fulminant DIC. Low fibrinogen levels have been advocated as a useful diagnostic marker of DIC; however, plasma levels may remain normal for prolonged periods despite ongoing consumption as fibrinogen also acts as an acute-phase reactant (5). Up to 57% of patients with DIC may have normal fibrinogen levels (6). CONCLUSIONS: As early recognition and treatment of PF is vital to reduce mortality, this case provides an important reminder that clinicians should not be deterred in considering a diagnosis based on an atypical feature or laboratory value. Reference #1: 1. Ahmed M, Samotowka M, Habis S, Mahmoud A, & Saeed R (2018). Escherichia coli Bacteremia-induced Purpura Fulminans: A Case Report. Cureus. Reference #2: 2. Lécuyer H, Borgel D, Nassif X, & Coureuil M (2017). Pathogenesis of meningococcal purpura fulminans. Pathogens and Disease, 75(3). 3. Ichimiya M, Takita Y, Yamaguchi M, & Muto M (2007). Case of purpura fulminans due to septicemia after artificial abortion. The Journal of Dermatology, 34(11), 786–789. Reference #3: 4. Huemer G M, Bonatti H, & Dunst K M (2004). Purpura fulminans due to E. coli septicemia. The Central European Journal of Medicine, 116(3), 82–82. 5. Levi M, Toh C H, Thachil J, & Watson H G (2009). Guidelines for the diagnosis and management of disseminated intravascular coagulation. British Journal of Haematology, 145(1), 24–33. 6. Spero J, Lewis J, & Hasiba U (1980). Disseminated Intravascular Coagulation. Thrombosis and Haemostasis, 43(01), 28–33. DISCLOSURES: No relevant relationships by Lehman Godwin, source=Web Response No relevant relationships by Jenny Han, source=Web Response Previous consultant in 2017 relationship with Third Rock Ventures Please note: $20001 - $100000 Added 01/03/2020 by Stephen Linderman, source=Web Response, value=Consulting fee Removed 01/03/2020 by Stephen Linderman, source=Web Response Previous consultant 2017-2018 relationship with Third Rock Ventures Please note: $20001 - $100000 Added 01/03/2020 by Stephen Linderman, source=Web Response, value=Consulting fee No relevant relationships by Douglas Parker, source=Web Response No relevant relationships by Jeeyon Rim, source=Web Response