Purpura fulminans

Background

• Life-threatening syndrome of intravascular coagulation and hemmorrhagic skin infarction--> DIC and vascular collapse
• Usually occurs in children, but can occur in adults
• Most commonly in the setting of overwhelming sepsis OR 7-10 days after infection
• Causes:
  • Meningococcus
  • Streptococci
  • Varicella
  • Rickettsial infections
  • Venom-induced consumptive coagulopathy (VICC) due to snakebite
  • Protein C, protein S, or antithrombin III deficiency

Clinical Features

• Erythematous macules rapidly progress--> Large, purpuric lesions
• DIC
• Hypotension
• Fever

Evaluation

• DIC work up
  • CBC, PT/PTT, fibrinogen, d-dimer, FDP
• Infectious work up

Management

• Treat shock
• Treat underlying infection
• Necrotic tissue may require debridement
• Coagulopathy correction, in some cases
  • Typically only if bleeding or need for procedure
    • Exception: FFP usually indicated for neonatal purpura fulminans
  • Fibrinogen: consider repletion with cryoprecipitate to raise level to 100-150
  • Platelets: consider repletion if <50K with bleeding or <20K without bleeding
  • FFP: consider repletion to goal of PT and PTT < 1.5 times the normal limit
  • Vitamin K, folate

Disposition

• Admit!

See Also

• Coagulopathy (Main)
• DIC