SESSION TITLE: Critical Care Student/Resident Case Report Posters III
SESSION TYPE: Medical Student/Resident Case Report
PRESENTED ON: Tuesday, October 29, 2013 at 01:30 PM - 02:30 PM
INTRODUCTION: Purpura Fulminans (PF) occurs in 15-25% of those with meningococcemia and carries a high mortality (50%). Early recognition and prompt treatment is essential to reduce morbidity and mortality.
CASE PRESENTATION: A 19-year old healthy female college student was transferred to our intensive care unit (ICU) from a local Emergency department (ED) for management of severe sepsis/septic shock and acute respiratory distress syndrome requiring intubation and mechanical ventilation. She presented to the local ED with fatigue, malaise, fever, and flu-like symptoms that progressed rapidly to hypotension, requiring rapid institution of early goal directed therapy. Laboratory data included leukocytosis, thrombocytopenia, and significant metabolic acidosis with a lactate of 10mmol/L. Skin exam revealed diffuse, maculopapular purpuric rash involving her trunk and extremities (Figure 1A and B) that progressed rapidly into black hemorrhagic skin necrosis and gangrene involving her toes and fingers (Figure 1C). She was on broad spectrum antibiotics. With the heightened suspicion of purpura fulminans (PF) secondary to meningococcemia, Vancomycin and Ceftriaxone were initiated. Blood cultures and emergent cerebrospinal fluid (CSF) analysis confirmed Neisseria Meningitidis. Her course was complicated by disseminated intravascular coagulation (DIC) necessitating platelets, fresh frozen plasma, and cryoprecipitate transfusions; adrenal insufficiency treated with hydrocortisone and fludrocortisone, renal failure requiring dialysis. She developed upper extremity compartment syndrome requiring bilateral forearm fasciotomies revealing necrotic tissue (Figure 2A). She required multiple toe amputations and skin grafts to areas affected by hemorrhagic necrosis and non-healing wounds (Figure 2B), and eventually recovered.
DISCUSSION: PF is a severe complication of meningococcemia occurring 12-24h after symptom onset. PF develops in the distal extremities and spreads proximally rapidly, leading to shock in 60-80% of cases. It is distinguished by acute onset of cutaneous hemorrhage with necrosis due to vascular thrombosis and DIC, ecchymoses that evolve into painful, indurated, well-demarcated papules surrounded by erythema. These papules evolve into bullae and vesicles with gangrenous necrosis. The differential diagnosis for our patient’s PF includes Henoch Schönlein purpura, post-infectious thrombocytopenic purpura, thrombotic thrombocytopenic purpura, and idiopathic purpura fulminans.
CONCLUSIONS: Heightened suspicion for PF secondary to meningococcemia is essential in patients presenting with diffuse maculopapular rash in the setting of severe sepsis/septic shock. Early recognition and treatment is essential to reduce morbidity and mortality.
Reference #1: Chalmers E, Cooper P, Forman K, et al. Purpura fulminans: recognition, diagnosis and management. Arch Dis Child 2011; 96:1066-71.
Reference #2: Betrosian AP, Berlet T, Agarwal B. Purpura Fulminans in Sepsis. Am J Med Sci 2006;332(6):339-345
DISCLOSURE: The following authors have nothing to disclose: Brittany Dykstra, Kannan Ramar
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