Purpura fulminans (PF) represents skin necrosis caused by intravascular necrosis and is often associated with vascular collapse and disseminated intravascular coagulation. Pf is usually precipitated by infection, drug use and/or deficiency of anticoagulants such as protein S or C. Typical infections include meningococcus, staphylococcus, streptococcus, and certain rickettsial infections. Chickenpox may precipitate PF. Presentation in the neonate is associated with hereditary deficiency of protein S, protein C and antithrombin III.
Sudden development of widespread purpura and necrosis is typical. Neonatal PF usually presents within several days of birth. The infant develops larger purpuric lesions over the trunk and proximal extremities. Protein C mutations or inherited deficiency of protein S or antithrombin III may be causative.
Post infectious PF typically develops 1-3 weeks after the precipitating infectious episode. Young children are most commonly affected and typically after varicella or streptococcal infections. After seeming to recover from the infection, the child develops larger purpuric lesions often of the buttocks and legs. The pathogenesis involves acute transient decrease in protein C, protein S, or antithrombin III levels.
Admission to the hospital and treatment by a specialist, e.g. infectious disease, internist is in order.
Erythematous area with necrosing center.
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