Purpura fulminans

Purpura fulminans
Classification and external resources
Specialty	hematology
ICD-10	D65 (ILDS D65.x20)
ICD-9-CM	286.6
DiseasesDB	34463
MeSH	D011695
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Purpura fulminans (also known as "Purpura gangrenosa") is an acute, often fatal, thrombotic disorder which manifests as blood spots, bruising and discolouration of the skin resulting from coagulation in small blood vessels within the skin and rapidly leads to skin necrosis and disseminated intravascular coagulation.

Purpura fulminans is rare and most commonly occurs in babies and small children but can also be a rare manifestation in adults when it is associated with severe infections. For example, Meningococcal septicaemia is complicated by purpura fulminans in 10–20% of cases among children. Purpura fulminans associated with congenital (inherited) protein C deficiency occurs in 1:500,000–1,000,000 live births.

Purpura fulminans is caused by defects in the protein C anticoagulant pathway. Identification of the cause of purpura fulminans often depends on the patient’s age and circumstances of presentation.

Congenital (inherited) defects in protein C activity are autosomal dominant and may be partial or severe loss of function. Hundreds of natural mutations of the protein C gene (PROC) have been identified.

Acquired protein C deficiency is caused by either depletion of available protein C in plasma or decreased protein C synthesis (caused by administration of vitamin k antagonists, severe liver failure or complications of prematurity).

Purpura fulminans is a presenting feature of severe acute sepsis, such as Neisseria meningitidis, , Group A and B , and less commonly with Haemophilus influenzae, Staphylococcus aureus, or Plasmodium falciparum (malaria) infections, particularly in individuals with asplenia.

In some cases, a combination of sepsis and a partial congenital defect in the protein C anticoagulant pathway initiates purpura fluminans.

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