Purpura fulminans (PF) is a rare vascular disorder that is characterized by fever, shock, multiorgan failure, and the rapid development of hemorrhagic skin necrosis. It is associated with dermal vascular thrombosis resulting from vascular collapse and disseminated intravascular coagulation (DIC). Purpura fulminans can result from hereditary or acquired protein C deficiency, activated protein C resistance, or protein S deficiency. It may also result from any condition that causes DIC.
Purpura fulminans presents with the dermatologic triad of widespread ecchymoses, hemorrhagic bullae [Fig 241-11 (Plate. . . 37)], and epidermal necrosis. Commonly, cyanosis with initial ecchymoses and ultimate necrosis of the tip of the nose, ears, and genitalia frequently occur; in general, distal tissue areas with end-circulation are affected. Large confluent ecchymoses can develop, often on the extremities—from distal to proximal—as well as on the perineum, buttocks, and abdomen. The extremities are often involved symmetrically. Treatment is directed at the underlying cause.
FIG. 241-11 (P|ate 37). Purpura fulminans with hemorrhagic bullae. (Reprinted with permission from Tangoren IA et al: Ecchymoses and epidermal necrosis in a 75-year-old man with bacteremia. J Crit Illness 13:108, 1998.)
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