Prompt recognition and specific therapy are crucial for better patient outcomes in managing Purpura Fulminans.
Understanding the role of protein C in the pathophysiology of Purpura Fulminans is crucial for targeted therapy.
Deep dives
Understanding Purper Fulminans
Purper Fulminans is a form of disseminated intravascular coagulation (DIC) characterized by profound microvascular thrombosis. Unlike other types of DIC, purper fulminans is marked predominantly by microvascular thrombosis in the skin, leading to skin necrosis. It is important to differentiate purper fulminans from other conditions like toxic shock syndrome and generic septic shock, as it requires specific management. Prompt recognition and specific therapy are crucial for better patient outcomes.
Types and Causes of Purper Fulminans
There are three types of purper fulminans - congenital, idiopathic post-infectious, and acute infectious. The congenital type manifests shortly after birth due to deficiencies in protein C, protein S, or antithrombin. The idiopathic post-infectious form occurs due to depletion of protein C activity by autoantibodies. The most common type is the acute infectious form, which occurs in patients with DIC where protein C becomes exhausted, resulting in excessive clotting. Identifying the specific type of purper fulminans is essential for appropriate treatment.
Pathophysiology and Role of Protein C
Protein C is an endogenous anticoagulant that plays a crucial role in balancing the coagulation process. In purper fulminans, a deficiency of protein C disrupts the balance between procoagulant and anticoagulant factors, leading to excessive clotting. Protein C helps break down factors V and VIII and promotes fibrinolysis, maintaining a balance in coagulation. In purper fulminans, protein C becomes depleted during septic shock, leading to exuberant clotting. Understanding the role of protein C in the pathophysiology of purper fulminans is crucial for targeted therapy.
Clinical Presentation and Diagnosis
Recognizing the clinical presentation of purper fulminans is important for timely diagnosis. Patients often present with non-blanching purpura, skin necrosis, and development of necrotic skin areas. Thrombosis may also occur in the adrenal glands, leading to adrenal insufficiency, and renal failure can occur. Differential diagnoses include catastrophic antiphospholipid antibody syndrome, hereditary protein C deficiency, and other causes of skin necrosis. Diagnostic tests typically show evidence of disseminated intravascular coagulation, including elevated INR, PTT, elevated D-dimer, and reduced protein C levels. While a skin biopsy may be performed to evaluate vasculitis, it is not always necessary for diagnosis.
In this episode we continue on the path of DIC: the scary sub-phenotype that is Purpura Fulminans. When your patient is necrosing everything, you need to act quickly before they start losing organs and digits. Come listen for identification, anticoagulation, protein C and all its goodness, when to give Vitamin K, and why to really be careful about Warfarin.
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