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Protein C deficiency

Kenneth A Bauer, MD
Section Editor
Lawrence LK Leung, MD
Deputy Editor
Jennifer S Tirnauer, MD


Protein C deficiency is associated with a small percentage of cases of inherited thrombophilia, as well as the even more uncommon findings of warfarin-induced skin necrosis and neonatal purpura fulminans, and a possible weak association with pregnancy loss. Despite these associations with adverse outcomes, hereditary protein C deficiency is uncommon, and diagnosis may be difficult in the setting of an acute thrombosis or anticoagulant administration. Management of individuals with protein C deficiency also raises several challenges.

This topic review discusses the diagnosis and management of protein C deficiency (inherited and acquired).

Separate topic reviews address the appropriate use of thrombophilia testing in various clinical settings:

Children – (See "Screening for inherited thrombophilia in children".)

Patients with venous thromboembolism – (See "Evaluating adult patients with established venous thromboembolism for acquired and inherited risk factors".)

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Literature review current through: Nov 2017. | This topic last updated: Nov 21, 2017.
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