Abstract

Purpura fulminans is a rare condition characterized by disseminated intravascular coagulation (DIC) with tissue thrombosis and skin necrosis occurring in approximately 10-20% of patients with meningococcemia. Diagnosis and management can be challenging given the infrequency of this clinical presentation. We present a case of Neisseria meningitides associated purpura fulminans that initially presented as acute hypoxic respiratory failure suspected to be secondary to Coronavirus Disease 2019 (COVID-19). A 55-year-old male with past medical history of hypertension presented to the emergency room after being tested for COVID-19 with fever, cough, and generalized weakness. At time of initial presentation, the patient was tachycardic and mildly febrile. Initial laboratory studies noted white blood cell count 2.5, platelets 127, lactate 2.3, potassium 3.3, and total bilirubin 1.7. Initial electrocardiogram and chest x-ray were unremarkable. Given suspicion for COVID-19, he was started on intravenous fluids, antibiotics, and admitted for further monitoring. Four hours later, he became persistently febrile, tachypneic, and hypoxic. Upon transfer to the Intensive Care Unit, a CTA pulmonary was obtained which showed dependent lung densities consistent with atelectasis. Repeat laboratory studies noted lactate 8.7, fibrinogen 131, ferritin 936, d-dimer > 4, procalcitonin > 40, and an upward trend in PT/INR. Ultimately, the patient was intubated for respiratory muscle fatigue. Post-intubation, he remained hypotensive and vasopressors were initiated. Later that afternoon, his COVID-19 test resulted as negative. During evening rounds, the patient developed significant bleeding from his oropharynx. Repeat investigation revealed platelet count 20, fibrinogen < 94, PT 26.8, and INR 2.37. He was transfused with fresh frozen plasma and cryoprecipitate. Admission blood cultures then grew gram-negative rods which speciated Neisseria meningitidis bacteria. Over the next few days, he developed significant skin necrosis. Plastic surgery was consulted and recommended transfer to a burn center. At the outside facility, given the extent of necrosis, the decision was made to pursue amputation. Subsequently, the patient underwent bilateral guillotine disarticulation of the wrists and bilateral below the knee amputations. In patients with DIC, sepsis, and an evolving skin rash, clinicians should have a high index of suspicion for purpura fulminans. Although initial presentations begin with small areas of petechiae or purpura, the onset of skin necrosis is swift. Delays in initiation of broad-spectrum antibiotic therapy place patients at an increased risk of mortality and limb loss. In this case, early diagnosis proved challenging given the patient's non-specific presenting symptoms in the absence of clinical evidence of meningitis.

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