What is your differential diagnosis for this patient? What tests would you send and what would you do next?

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Final Diagnosis: Purpura Fulminans due to Streptococcus pneumoniae bacteremia



Case Conclusion: The patient was treated with broad spectrum antibiotics and aggressively resuscitated. Lab work revealed thrombocytopenia, low fibrinogen, coagulopathy, and elevated d-dimer consistent with disseminated intravascular coagulation (DIC). The patient went into mult-system organ failure requiring ventilatory support, dialysis and vasopressors. Blood cultures grew Strep pneumoniae. It was determined that the patient's major risk factor for this infection was congenital asplenia (see CT scan above), and she was vaccinated against Neissieria and Pneumococcus after recovery and prior to discharge from the hospital.

Learning Points: Purpura Fulminans(PF) is the result of an acute inflammatory response early in sepsis and is most commonly seen in pediatric or younger patients [1]. In sepsis, it is a clinical manifestation of DIC in which widespread activation of the coagulation pathway and consumption of anticoagulant proteins (especially Protein C) leads to widespread microvascular thrombosis. Microvascular thrombosis in the skin is indicative of a systemic process, and affected patients are likely to also have hemorrhagic infarction in other organs, especially the lungs, kidneys, central nervous system and adrenal glands. The development of PF is therefore a harbinger of impending multi-system organ failure. Mortality rate in patients with PF exceeds 40% [2].