THROMBO-HEMORRHAGIC SKIN NECROSIS DUE TO RAPID DEVELOPMENT OF SEVERE VITAMIN K DEFICIENCY ASSOCIATED WITH CHOLESTASIS

A female patient is presented, who developed thrombotic and hemorrhagic skin necrosis of the feet and toes during an acute episode of severe vitamin K deficiency due to cholestasis in the absence of coumarin treatment. Painful blue toes and feet progressed to erythematous swelling and bluish discoloration with blister formations and immanent gangrene. The histo-pathology of skin excisions from the erythematous skin lesions showed extravasation of erythrocytes and extensive fibrin thrombus formations in capillaries and venules as has been described in patients with coumarin skin necrosis. At the time of painful acrocyanosis the results of coagulation investigations (platelets 109 × 10⁹/1, APTT 56/35 sec., PT 61/15 sec., Thrombotest^R (TT) less than 3%, Normotest^R (NT) less than 10%, fibrinogen 1,6 g/l, absence of fibrin monomers and degradation products, factor V 1.00 u/ml, antithrombin lil 1.03 u/ml and alfa₂ antiplasmin 0.97 u/ml were consistent with severe vitamin K deficiency. Measurements of vitamin K dependent factors revealed very low levels for procoagulant factor VII (16%) and protein C (22%) antigen concentration and normal levels for procoagulant factor II (97%) and procoagulant factor X (87%) antigen concentration. After substitution of 5 mg vitamin K1 both the TT and NT normalized.These data confirm the hypothesis that an imbalance within procoagulant and anticoagulant vitamin K dependent factors (severe protein C and factor VII deficiency as compared to the procoagulant factors IX, X and II due to rapid development of vitamin K deficiency) contributes to the pathogenesis of thrombo-hemorrhagic skin necrosis and that the so-called coumarin necrosis is not due to drug toxicity.