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Purpura Fulminans, Pediatric

Mihir D. Bhatt, MD, FRCPC and Victoria E. Price, MBChB, MSc, FRCPC Reviewed 10/2018
 


BASICS

DESCRIPTION

  • Hematologic emergency characterized by dermal hemorrhagic necrosis and disseminated intravascular coagulation (DIC)

  • Associated with a congenital or acquired protein C and/or protein S...

DIAGNOSIS

HISTORY

  • 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...

TREATMENT

GENERAL MEASURES

  • 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 ...

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

Patient Monitoring

  • When to expect improvement: related to underlying cause of purpura fulminans

  • Signs to watch for:

    • Spread of purpura

    • Hypotension

    • Gangrene

  • Treatment dur...

ADDITIONAL READING

  • 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...

CODES

ICD9

  • 286.6 Defibrination syndrome

  • 772.6 Cutaneous hemorrhage of fetus or newborn

ICD10

  • D65 Disseminated intravascular coagulation

  • P54.5 Neonatal cutaneous hemorrhage

SNOMED

  • 13507004 Purpura fulminans (...

FAQ

  • 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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