Purpura fulminans: Difference between revisions
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==Differential Diagnosis== | ==Differential Diagnosis== | ||
{{Purpura DDX}} | |||
==Evaluation== | ==Evaluation== |
Revision as of 02:04, 15 August 2016
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
Differential Diagnosis
Petechiae/Purpura (by cause)
- Abnormal platelet count and/or coagulation
- Septicemia
- Idiopathic thrombocytopenic purpura (ITP)
- Hemolytic uremic syndrome
- Leukemia
- Coagulopathies (e.g. hemophilia)
- Henoch-Schonlein Purpura (HSP)
- Acute hemorrhagic edema of infancy (AHEI)
- Hypersensitivity vasculitis
- Primary vasculitides
- Wegener's
- Microscopic polyangiitis
- Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)
- Secondary vasculitides
- Trauma
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
- Typically only if bleeding or need for procedure
Disposition
- Admit!