Purpura Fulminans
Epidemiology
History : first described by Guelliot in 1884
Etiology
Hemostasis-Initiated Purpura Fulminans
Acute Infectious Purpura Fulminans
Post-Infectious/Idiopathic Purpura Fulminans
Clinical Presentations
Neonatal Purpura Fulminans
Epidemiology : usually associated with protein C, protein S, or anti-thrombin III deficiencyDiagnosis : decreased protein C, protein S, or anti-thrombin IIIClinical : occurs within the first 72 hrs after birth
Acute Infectious Purpura Fulminans
Post-Infectious/Idiopathic Purpura Fulminans
Epidemiology Most cases occur in children Over 90% of cases are preceded by infection Physiology : bacterial endotoxin -> imbalance in anticoagulant and procoagulant endothelial cell function, consumption in protein C, protein S, and anti-thrombin III, micromeboli, and direct bacterial damageDiagnosis : severely decreased protein C, protein S, and anti-thrombin IIIClinical : onset usually 7-10 days after the precipitating infectionLarge ecchymoses with sharply demarcated irregular shapes -> evolve into hemorrhagic bullae -> black necrotic lesions and gangrene May involve heart, kidneys, and lungs
Treatment
Neonatal Purpura Fulminans
Acute Infectious Purpura Fulminans
Antibiotics : especially to cover for Neisseria and MRSAActivated Protein C Intravenous Immunoglobulin (IVIG) (see Intravenous Immunoglobulin ): contains antibodies against the causative exotoxinsSurgical Debridement Hyperbaric Oxygen (see Oxygen ): probably not beneficial
Post-Infectious/Idiopathic Purpura Fulminans
Antibiotics Surgical Debridement Treatment of Compartment Syndrome : if presentActivated Protein C Alteplase (see Alteplase )
References
Capnocytophaga canimorsus septicemia in Denmark, 1982-1995: review of 39 cases. Clin Infect Dis 1996; 23(1):71-75 [MEDLINE ] Purpura fulminans due to Staphylococcus aureus. Clin Infect Dis. 2005 Apr 1;40(7):941-7 [MEDLINE ] Capnocytophaga canimorsus sepsis. Blood 2010; 116(9):1396 [MEDLINE ] A Case of Purpura Fulminans Arising from Cryptococcosis. Am. J. Respir. Crit. Care Med. 2012; 186: 109-110
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