Purpura fulminans (PF) is an uncommon syndrome characterized by infarction, hemorrhage and thrombosis. It is, at its core, coagulation gone astray that occurs in association with infection and sepsis. It is accompanied by shock, hypotension and acidosis. It is characterized by DIC and the appearance of visible purpura. All of this results in the necrosis of skin, soft tissue, muscle and bone. This is an important distinction because in many of the reports on PF, the implication is that it is a skin and soft tissue problem alone, but it can result in bony infarction as well.
The pathophysiology is a release of bacterial endotoxin and activation of inflammatory cytokines. There is consumption or, in fact, intrinsic deficiency of the endogenous anticoagulants - protein C, protein S and antithrombin III. The result is small vessel thrombosis, multisystem organ failure and a high mortality.
The signs and symptoms are fairly protean, often mimicking a flu-like syndrome. The symptoms could be those of an upper respiratory infection or a gastroenteritis, however it is commonly associated with a high fever and occasionally emesis. Finally, after the appearance of a rash, medical attention is sought. These children can exhibit lethargy, sometimes seizures, respiratory distress and progression to vascular collapse.