Livedoid Vasculitis With Ulcerations: The Role of Antithrombin III Deficiency and Its Therapeutic Consequences

Livedoid Vasculopathy

“…It has been described as idiopathic, 5 associated with immune complexassociated diseases, 4 and a result of dermal blood vessel occlusion. 6 Laboratory test result abnormalities have been reported in patients with the disease, including defective release of tissue plasminogen activator, 7 elevated fibrinopeptide A, 8 antithrombin III deficiency, 9 and numerous other thrombophilic abnormalities that are further described in this article. The purpose of our review was to further characterize the clinical features, disease associations, and laboratory test result abnormalities, including coagulation study results, in patients with biopsy-proved livedoid vasculopathy whose conditions were evaluated at the Mayo Clinic, Rochester, Minn, from January 1,1990, to December 31, 2000.…”

Frequency of thrombophilia determinant factors in patients with livedoid vasculopathy and treatment with anticoagulant drugs – a prospective study

“…There is, however, increasing evidence that the condition must be mediated by either coagulation or fibrinolysis disorders 7–10 . Some reports have described its association with inadequate fibrinolytic processes, platelet dysfunction, increase in tissue plasminogen activator (tPA) inhibitor levels, 11 cryofribrinogenaemia, 12 hyperhomocysteinaemia, 12 antithrombin III deficiency, 9 factor V Leiden mutation, 13 and antiphospholipid antibodies 14 . Positive clinical response to fibrinolytic and antiplatelet drugs, and to anticoagulation therapies, is an additional evidence of the role of coagulation disorders in LV’s pathogenesis.…”

Mononeuropathy multiplex in association with livedoid vasculitis

“…Pathological evidence of thrombosis was found within the vascular lesions of livedoid vasculitis, and this could be due to a number of abnormalities within the thrombotic and fibrinolytic systems. Associations with livedoid vasculitis have included abnormal serum antiphospholipid antibodies,1, 14 protein C deficiency,2, 4 abnormalities of the tissue plasminogen activator (tPA) system,29 elevated homocysteine levels,10 antithrombin III deficiency,15 and factor V Leiden mutations 5. These studies suggest that the thrombosis found to occur in livedoid vasculitis is part of a primary pathophysiological mechanism.…”