Livedoid vasculopathy secondary to high levels of lipoprotein(a)

Abstract

Funding sources: none. Conflicts of interest: none declared. Madam, Livedoid vasculopathy (LV) was first described by Bard and Winkelmann in 1967.1 It is characterized by a long history of recurrent painful ulcerations of the feet and legs2 and scar lesions named atrophie blanche.3 LV is a thrombotic (occlusive) vasculopathy of the small vessels of the lower extremities.3 Histology shows microthromboses and/or segmental hyalinization of the subendothelial intimal layer of blood vessels of the middle and lower dermis.3 Its exact aetiology remains uncertain, but thrombotic and microcirculatory phenomena have been implicated as possible pathogenic factors.3, 4 Several thrombophilic conditions have been reported to be associated with LV (Table 1).2–7 We report two patients with LV secondary to high levels of lipoprotein(a) [Lp(a)]. Patient 1 was a 29‐year‐old white woman, referred to us because of recurrent painful ulcerations in the legs associated with extensive livedo racemosa on her upper and lower limbs. There were several shallow ulcerations in the malleolar regions, necrosis and crusts (Fig. 1). She had previously been treated with oral aspirin and pentoxifylline without response. Skin biopsy confirmed the clinical diagnosis of LV. Results of extensive laboratory testing for the established abnormalities associated with LV were all negative, including thrombophilic states [factor V G1691A gene mutation (Leiden), prothrombin G20210A gene mutation, methylenetetrahydrofolate reductase (MTHFR) mutation and homocysteinaemia, protein C and/or protein S deficiency, antithrombin III deficiency, anticardiolipin antibodies, lupus anticoagulant], hepatitis B and C infection and autoimmune diseases. We introduced oral warfarin and international normalized ratio (INR) was maintained at 3. One week after the treatment onset, the pain ceased. Total healing of the cutaneous ulcerations was obtained after the third month under warfarin therapy. After this, ulcerations relapsed twice, but on both occasions the INR was below the therapeutic level (2–3) and the patient quickly responded after that level was reached. Because of the recent description of an association between LV and elevated levels of Lp(a),6 we reviewed the case and found an increased level of Lp(a) (51 mg dL−1; normal < 30).