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

Abstract

The authors suggest all patients with diagnosis of LV to be investigated for thrombophilic status. Anticoagulant drugs were well tolerated and seemed to be effective in treating not only LV symptoms but also its ulcerations.

Vasculopatia livedoide: uma doença cutânea intrigante

“…A physical examination may reveal lesions in different stages of evolution, usually associated with livedo racemosa. 8 During its evolution, purpuric and erythematous papular plaques and papules are formed. These plaques and papules are painful on palpation and sometimes initially itchy.…”

“…They may also coalesce into large ulcers in a geographical form, which heal slowly, over about 3 to 4 months, with ivory-white stellate borders and atrophic center, presenting the morphological appearance of White Atrophy (Figures 1 and 2). 5,8 Peripheral punctate telangiectasias and slough appear in some areas that are about to ulcerate. 5 The lesions usually occur symmetrically on the legs and feet.…”

“…Some examples of hereditary thrombophilias are deficiencies of natural anticoagulants (antithrombin, protein C, protein S and protein Z), prothrombin mutation, factor V (Leiden) mutation, hyperhomocysteinemia (due to genetic enzyme defect) and changes in the plasma levels of coagulation factors (VIII, IX and XI), in addition to deficiencies of the fibrinolytic system ( Figure 4). 8,18 Other associations related to the etiopathogenesis of LV are the presence of high levels of plasminogen activator inhibitor-1 (PAI-1) and increased levels of lipoprotein(a). 19,20 Table 1 summarizes possible etiopathogenic associations in LV.…”

“…21 Among the several reported associations between LV and hypercoagulable states are the following: protein C deficiency, factor V (Leiden) mutation, prothrombin gene mutation, hyperhomocysteinemia, increased levels of lipoprotein(a) and antithrombin deficiency. 8,9,14,20,[30][31][32][33][34][35][36] Consequently, given the heterogeneity of the etiopathogenic factors clinically involved in LV, we propose to classify it according to its etiopathogenesis into primary (idiopathic) or secondary to a known event or condition, with the latter being subdivided into four groups: (i) endothelial damage, (ii) changes in blood flow, (iii) states related to thrombophilias, and (iv) mixed etiopathogenesis.…”

“…It especially affects the age group between 15 and 50 years old (mean 32 years) and females at a proportion of 2.4 to 3 women for every man. [6][7][8][9] LV was originally described as a clinical manifestation of vasculitis. However, at present, the main physiopathogenic mechanism considered is a vasoocclusive phenomenon due to intraluminal thrombosis of dermal venules 1 .…”