Digital preservation following purpura fulminans: A case report Free

Background Disseminated intravascular coagulation (DIC) is a complex, life-threatening disorder characterized by systemic activation of coagulation pathways resulting in thrombocytopenia, microvascular thrombosis, and a risk of severe hemorrhage. Purpura fulminans (PF) is an uncommon but severe complication, particularly in sepsis-associated DIC, manifesting with extensive cutaneous hemorrhagic infarctions, rapidly progressing to large vessel thrombosis and multi-organ failure. Due to the bleeding risk, anticoagulation is traditionally avoided in DIC. We report a case of purpura fulminans-associated DIC with marked clinical and laboratory improvement following therapeutic anticoagulation.

Case Presentation A 44-year-old previously healthy female presented to the emergency department with severe nausea and vomiting following seafood intake. Vital signs on admission were: blood pressure 90/50 mmHg, heart rate 124 bpm, and she was febrile with Tmax of 103.8F. On examination, she appeared ill, exhibited mottling and cyanosis of bilateral fingers and toes, with facial petechiae. Laboratory investigations revealed a white blood cell count of 31k/uL, hemoglobin 10.9g/dL, and thrombocytopenia with platelet count of 30 × 10⁹/L. Blood cultures grew Streptococcus pneumoniae. Complement levels were within normal limits. She required intensive care admission for pressor support. Empiric intravenous antibiotics were initiated in consultation with infectious disease specialists. Her clinical course was complicated by sepsis-induced cardiomyopathy and acute kidney injury. Notably, imaging identified an atrophic spleen.

Coagulation studies demonstrated a markedly deranged profile: PT 30 seconds, PTT 116.3 seconds, INR 2.5, fibrinogen 103 mg/dL, and D-dimer >50,000 ng/mL. Protein S was 48% and protein C 82%. Peripheral blood smear showed significant schistocytosis, consistent with severe DIC and purpura fulminans.

Given the worsening cyanosis of digits of her hands and feet, and no evidence of bleeding, she was cautiously started on low dose heparin infusion (20,000U over 24 hours) with initial 5000u intravenous loading dose. She also received fresh frozen plasma and platelet transfusion to keep platelet>50 × 10⁹/L. Within 24 hours of anticoagulation initiation, platelet counts increased, D-dimer levels decreased to 16,000 ng/mL, PT/PTT/INR normalized, and fibrinogen rose to 289 mg/dL. Heparin dosage was escalated to therapeutic range accordingly. After 48 hours, improvement in peripheral cyanosis and petechiae was noted clinically, with continued favorable laboratory trends.

By day six, coagulation parameters normalized fully, and necrotic changes on the extremities began to regress with improved perfusion and reduced demarcation of ischemic areas. She was discharged on prophylactic heparin dose and plans for vaccination against capsulated organisms, with outpatient follow up. Outpatient hematology and vascular follow-up demonstrate significant wound healing with only superficial eschars on distal toes, without warranting any surgical intervention.

Discussion

This case highlights the pivotal role of early therapeutic anticoagulation in altering the trajectory of purpura fulminans-associated DIC. PF is characterized by rapid microvascular thrombosis leading to extensive hemorrhagic necrosis within 24–48 hours, often culminating in the need for surgical debridement or amputation, with significant morbidity and mortality.

DIC precipitates a consumptive coagulopathy with paradoxical coexistence of systemic thrombosis and bleeding risk due to depletion of clotting factors and platelets. As a result, anticoagulation is typically contraindicated. However, certain phenotypes of DIC, particularly the highly prothrombotic purpura fulminans subtype, may benefit from cautious anticoagulation combined with factor repletion. In our patient, early initiation of low-dose heparin within 48 hours of symptom onset was associated with rapid reversal of laboratory derangements, clinical resolution of ischemic necrosis, and preservation of limb function. This underscores the importance of timely anticoagulation to interrupt microvascular thrombosis and facilitate reperfusion.