Introduction: Purpura fulminans is a rare rapidly-progressive thrombotic disorder characterized by disseminated intravascular coagulation (DIC), tissue necrosis, and multiorgan failure. It is precipitated by severe bacterial infection, especially in functionally asplenic or immunocompromised patients. Though most commonly associated with meningococcemia, Streptococcus pneumoniae is also a known inciting pathogen.
Description: A 36-year-old man with known sickle cell trait and undiagnosed asplenia was found unresponsive after two days of rigors and poor sleep. His children were recently sick with fever and cough. He was febrile to 105.2°F and encephalopathic with laboratory studies notable for leukocytosis, lactate (9.8 mmol/L), coagulopathy, acute kidney injury, and transaminitis. Imaging revealed right lower lobe consolidation and evidence of autosplenectomy. Blood cultures grew Streptococcus pneumoniae. Despite broad-spectrum antibiotics, his condition deteriorated with rising creatinine (5.2 mg/dL) and rhabdomyolysis (CK >59,000 U/L) prompting CRRT initiation. He developed a diffuse purpuric rash and laboratory features consistent with DIC: thrombocytopenia, prolonged PTT, schistocytes, and low fibrinogen. Arterial dopplers showed loss of distal perfusion with compartment syndrome suspected in all extremities. Fasciotomy was deferred due to instability. Unfortunately, the patient suffered cardiac arrest in the setting of refractory hyperkalemia, shock, and multiorgan failure.
Discussion: This case highlights the severity of purpura fulminans due to pneumococcal sepsis in a patient with sickle cell trait. The combination of functional hyposplenism in the absence of prior vaccination, systemic inflammation, and hypercoagulability created a prothrombotic storm unresponsive to standard interventions. Early recognition of purpura fulminans is critical to provide optimal antimicrobial coverage to improve outcomes. Acquired protein C deficiency may contribute to the pathophysiology of this condition; although this patient received plasma without notable improvement, protein C replacement therapy may be beneficial in some cases. Optimal vaccination, prompt antibiotic therapy, and aggressive DIC management remain the cornerstones of prevention and care in such high-risk populations.
