Severe Acquired Protein C Deficiency in Purpura Fulminans Associated With Disseminated Intravascular Coagulation: Treatment With Protein C Concentrate

Abstract

Purpura fulminans (PF) defines an acute, often lethal syndrome of disseminated intravascular coagulation (DIC) with rapidly progressive hemorrhagic necrosis of the skin due to dermal vascular thrombosis.1-7 It is indicative of a severe disturbance in hemostasis now recognized to involve the protein C system in many cases.1,2,5,8-12 Purpura fulminans is usually seen in three clinical settings: (1) in the newborn period as a manifestation of homozygous protein C deficiency, or rarely protein S deficiency13,14; (2) in individuals with acute, severe viral or bacterial infection where an acquired deficiency in protein C activity is documented1-3,5-8,10,12,15; and (3) as a rare, postinfectious syndrome with a history of an antecedent" preparatory disease," most commonly a viral or bacterial illness involving the skin (eg, varicella or scarlet fever), with the sudden development, during an otherwise unremarkable convalescence, of progressive purpura and necrosis.3-5,7,10,12