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

External Links

References

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