Acute Infectious Purpura Fulminans: Pathogenesis and Medical Management

Abstract

Purpura fulminans (PF) is a potentially disabling and life-threatening disorder characterized by acute onset of progressive cutaneous hemorrhage and necrosis, and disseminated intravascular necrosis. Acute infectious PF occurs most commonly in the setting of meningococcemia due to elaboration of endotoxin. Presence of purpura, particularly when generalized, is an important predictor of a poor outcome following meningococcal infection. Histopathologic hallmarks of acute infectious PF are dermal vascular thrombosis and secondary hemorrhagic necrosis, findings which are identical to those of the Shwartzman reaction. Acute infectious PF and the Shwartzman reaction have a common pathogenesis, involving a disturbance in the balance of anticoagulant and procoagulant activities of endothelial cells. This disturbance, which is triggered by endotoxin, appears to be mediated by cytokines, particularly interleukin-12, interferon-gamma, tumor necrosis factor-alpha, and interleukin-1, leading to the consumption of proteins C and S and antithrombin III. State-of-the-art therapeutic interventions based on recent advances in our understanding of the pathogenesis of acute infectious PF are discussed.

Outcome in children with purpura fulminans: Report on 16 patients

“…A few reports describe PF in children, and most of the cases are related to infectious disease such as meningococcemia, varicella, or pneumococcal disease or to severe congenital PC or PS deficiency [1,5,11]. Congenital and acquired risk factors in childhood PF are largely unknown, and additionally, with effective immunization programs against viral infections and better health care provided to children, one may expect changes in the rate of participating infections in PF.…”

Purpura Fulminans with Peripheral Gangrene in Severe Falciparum Malaria: A Case Series

“…To the best of our knowledge, this is one of the earliest reports of falciparum malaria complicated with PF in an endemic malaria setting and involving children. Previous studies have shown that 60 to 70% cases of acute purpura fulminans occur amongst children below two years of age [13]. The two previous cases of PF reported previously in Nigeria involved a two month old and three year-old with symmetric gangrene.…”

“…Cumulatively these changes might explain the pathogenesis behind the thrombocytopenia and intravascular platelet aggregation. Transfusion of coagulation factor, protein C, protein S, antithrombin III concentrates, have been shown to be beneficial in patients with PF and DIC [1,13].…”

Case Report- Acute Infectious Purpura Fulminans Due to Klebsiella Pneumoniae

“…iii) Acute infectious purpura fulminans, occurs in conjunction with acute infectious illness, particularly sepsis with endotoxin (lipopolysaccharide) producing gram-negative bacteria. [2] Sepsis-induced purpura fulminans is a rare but life-threatening disorder. Meningococcal sepsis is the most common cause.…”