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
• Streptococcus species
• Varicella
• Rickettsia sp
• Venom-induced consumptive coagulopathy (VICC) due to snake bites
• Protein C, protein S, or antithrombin III deficiency
• Drug-induced

Clinical Features

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

Neonatal purpura fulminans

Differential Diagnosis

Petechiae/Purpura (by findings)

• Febrile, toxic
  • Palpable
    • Meningococcemia
    • Disseminated gonococcal infection
    • Endocarditis
    • Rocky mountain spotted fever
    • HSP
  • Non-palpable
    • Purpura fulminans
    • DIC
    • TTP
• Afebrile, nontoxic
  • Palpable
    • Autoimmune vasculitis
  • Non-palpable
    • ITP

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
  • Cryoprecipitate: consider if fibrinogen <100
  • 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

References

Authors

Claire, Ross Donaldson, Michael Holtz