An inherited deficiency of
protein C, a recognized hypercoagulable state, may cause a clinically significant
deep venous thrombosis. Only some persons with a deficiency of
protein C experience
thrombosis, and almost always the thrombotic event occurs in the venous circulation.
Warfarin-induced skin
necrosis, a rare event observed in some patients soon
after treatment with
warfarin is begun, is believed to be another manifestation of this deficiency. We describe a young woman whose basal functional and antigenic levels of
protein C were about 45% and who experienced both
deep venous thrombosis and
warfarin-induced skin
necrosis in a clinically severe course. Evidence for lupus
anticoagulants was present, with prolonged activated partial thromboplastin time that was corrected when lysed platelets were added, prolonged Russell's viper venom time,
anticardiolipin antibodies, and other laboratory evidence. Lupus
anticoagulants are associated also with a significant incidence of
thrombosis, including arterial
thrombosis, and this patient developed concurrently arterial
thrombosis. The combined effects of
protein C deficiency and lupus
anticoagulants, exacerbated by other potentially thrombogenic conditions, are believed responsible for the severe thrombotic events experienced by this patient.