Purpura fulminans

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.

Epidemiology

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.

Causes

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 protein C deficiency

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.