Purpura fulminans.

Purpura fulminans presents as a catastrophic illness with gangrene of the distal extremities and necrosis of skin. The clinical picture consists of septicemia, shock, and disseminated intravascular coagulation. The Shwartzman and Arthus reactions are thought to be responsible for the pathogenesis of purpura fulminans. The exact mechanisms of these reactions are not completely understood. Immediate resuscitation is the treatment for shock and sepsis. Heparin is recommended to reverse the disseminated intravascular coagulation component of this disease. Surviving patients require treatment of skin necrosis and digital and extremity gangrene. The former are managed in a fashion similar to the management of burns. Amputation should be delayed until maximal collateral circulation has developed. A series of 10 patients is presented and 58 cases from the literature are analyzed.