Hematologic emergency characterized by dermal hemorrhagic necrosis and disseminated intravascular coagulation (DIC)
Associated with a congenital or acquired protein C and/or protein S...
Current sepsis: fever, weakness, dizziness, nausea, vomiting, onset of petechial/purpuric rash
Recent history of febrile illness
Medications (e.g., warfarin)
Family history suggestive of...
If classical signs of purpura fulminans, therapy should be commenced emergently.
Treat underlying cause.
Anti-infective agents depending on underlying cause
Manage DIC based on ...
When to expect improvement: related to underlying cause of purpura fulminans
Signs to watch for:
Spread of purpura
Goldenberg NA, Manco-Johnson MJ. Protein C deficiency. Haemophilia. 2008;14(6):1214–1221. [View Abstract on OvidInsights]
Leclerc F, Leteurtre S, Cremer R, et al. Do new strate...
286.6 Defibrination syndrome
772.6 Cutaneous hemorrhage of fetus or newborn
D65 Disseminated intravascular coagulation
P54.5 Neonatal cutaneous hemorrhage
13507004 Purpura fulminans (...
Q: What is the risk of a second affected child with protein C or S deficiency?
A: If the diagnosis is confirmed by family studies that show both parents to be carriers of the deficiency and the aff...
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