Summary
We describe a 57-year-old woman with homozygous protein C deficiency and mild thrombotic
manifestations consisting of three spontaneous distal deep vein thromboses occurring
after the age of 45. Previous surgery and pregnancies had been uneventful. Low but
detectable protein C antigen and activity levels (both 20%) were discovered on the
occasion of skin necrosis induced by oral anticoagulation. This therapy was interrupted
because of skin necrosis and several episodes of disseminated intravascular coagulation
(DIC) at the initiation of treatment despite a cautious protocol. No recurrent thromboembolic
event has occurred in our patient using prophylactic doses of low molecular weight
heparin for 24 months. New therapeutic approaches might be the administration of low
molecular weight heparin or oral anticoagulation associated with protein C replacement
in the induction period. This case reflects the variability of expression of protein
C deficiency as well as the potential hazards of antivitamin K anticoagulation in
this disorder.
