Abstract Introduction Purpura fulminans (PF) is a rare, life-threatening thrombotic disorder characterized by sudden-onset, rapidly progressive cutaneous hemorrhage and necrosis due to dermal vascular thrombosis and disseminated intravascular coagulation. While acquired or congenital Protein C deficiencies can underlie PF, the most common triggers are infection, specifically Neisseria meningitidis and Streptococcus pneumoniae. Streptococcus pyogenes-induced PF is rare in immunocompetent adults and is associated with high morbidity and mortality. Early recognition is key, but even with best supportive care, limb loss is frequent amongst survivors. We discuss the case of a patient with S. pyogenes bacteremia who developed rapidly progressive, limb threatening PF. Description A 60-year-old male with no known medical history, but ongoing methamphetamine use, presented to the emergency department with altered mental status after being found down at home by family. Last known well was 48 hours prior to presentation. He was intubated and admitted. Laboratory evaluation was significant for leukocytosis (WBC 18.3 × 10³/μL) and thrombocytopenia (platelets 88 × 10³/μL). He also had rhabdomyolysis with elevated creatinine kinase (20,225 U/L), lactic acidosis (10.3 mmol/L) and acute kidney injury (creatinine 2.66 mg/dL). Two sets of blood cultures drawn on presentation rapidly grew S. pyogenes and the patient was initiated on vancomycin and piperacillin-tazobactam. The patient developed acute respiratory distress syndrome (ARDS) as well as septic shock requiring vasopressor support and emergent initiation of sustained low-efficiency dialysis. On hospital day three, well-defined, non-blanchable purpuric patches with central necrosis (Figure 1) were noted on bilateral lower and left upper extremities, consistent with PF. Urgent computerized tomography imaging and surgical consultation was initiated, but there was no indication of necrotizing soft tissue infection or trigger for surgical intervention. However, bilateral knee effusions were noted and arthrocentesis demonstrated a white blood cell count of 185,000 with fluid cultures positive for GAS. Discussion PF triggered by S. pyogenes is a distinctive and exceedingly rare manifestation of invasive GAS infection. Literature review identifies fewer than 15 reported cases to date, with most presenting with shock and coagulopathy and 50% mortality, despite otherwise healthy status. The underlying pathophysiology involves profound consumption of anticoagulants and widespread dermal microvascular thrombosis leading to hemorrhagic skin necrosis. Early recognition, prompt targeted antibiotic therapy and aggressive supportive care with anticoagulation and surgical debridement are critical to improving outcomes. In this case, septic arthritis was the likely source, and the patient underwent surgical debridement of his left knee, but remains critically ill. This abstract is funded by: None
Gerami et al. (Fri,) studied this question.