Purpura Fulminans

Epidemiology

  • History: first described by Guelliot in 1884

Etiology

Hemostasis-Initiated Purpura Fulminans

Acute Infectious Purpura Fulminans

Post-Infectious/Idiopathic Purpura Fulminans

  • Gastroenteritis (see Gastroenteritis, [[Gastroenteritis]])
  • Measles (see Measles Virus, [[Measles Virus]])
  • Neisseria Meningitidis (see Neisseria Meningitidis, [[Neisseria Meningitidis]]): common etiology (most common in infants and adolescents)
  • Rubella (German Measles) (see Rubella, [[Rubella]])
  • Scarlet Fever (se Scarlet Fever, [[Scarlet Fever]])
  • Streptococcal Pharyngitis (see Streptococcus, [[Streptococcus]]): Streptococcus species are the most common etiology in adults
  • Upper Respiratory Infection
  • Varicella (see Varicella-Zoster Virus, [[Varicella-Zoster Virus]]): common etiology

Clinical Presentations

Neonatal Purpura Fulminans

  • Epidemiology: usually associated with protein C, protein S, or anti-thrombin III deficiency
  • Diagnosis: decreased protein C, protein S, or anti-thrombin III
  • Clinical: occurs within the first 72 hrs after birth

Acute Infectious Purpura Fulminans

  • Epidemiology: most common type
  • Physiology: involves decreased protein S resulting in severe disseminated intravascular coagulation (DIC) (see Disseminated Intravascular Coagulation, [[Disseminated Intravascular Coagulation]])
  • Clinical

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 damage
  • Diagnosis: severely decreased protein C, protein S, and anti-thrombin III
  • Clinical: onset usually 7-10 days after the precipitating infection
    • Large 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

  • Fresh Frozen Plasma (FFP) (see Fresh Frozen Plasma, [[Fresh Frozen Plasma]]): to replace protein C
  • Low Molecular Weight Heparin/Coumadin (see Enoxaparin, [[Enoxaparin]] and Coumadin, [[Coumadin]]): after FFP

Acute Infectious Purpura Fulminans

  • Antibiotics: especially to cover for Neisseria and MRSA
  • Activated Protein C
  • Intravenous Immunoglobulin (IVIG) (see Intravenous Immunoglobulin, [[Intravenous Immunoglobulin]]): contains antibodies against the causative exotoxins
  • Surgical Debridement
  • Hyperbaric Oxygen (see Oxygen, [[Oxygen]]): probably not beneficial

Post-Infectious/Idiopathic Purpura Fulminans

  • Antibiotics
  • Surgical Debridement
  • Treatment of Compartment Syndrome: if present
  • Activated Protein C
  • Alteplase (see Alteplase, [[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