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Recalcitrant Livedoid Vasculopathy Associated with Hyperhomocysteinaemia Responding to Folic Acid and Vitamins B6/B12 Supplementation.

© 2016 The Authors. doi: 10.2340/00015555-2416 Journal Compilation © 2016 Acta Dermato-Venereologica. ISSN 0001-5555 Livedoid vasculopathy (LV) is a rare skin disease affecting especially the calves, ankles and dorsum of the feet, and usually presenting with painful, purpuric macules/ papules and small ulcerations evolving into livedoid brownish pigmentation and stellate scars (“atrophie blanche”) with surrounding punctate telangiectasias; a history of livedo reticularis may also be present (1, 2). Histologically, microthromboses and segmental hyalinization of the subendothelial intima of blood vessels of the middle/lower dermis are seen. LV is considered to be an occlusive vasculopathy due to a procoagulant state (1, 2), including protein C deficiency, factor V Leiden mutation, prothrombin gene mutation, increased levels of plasminogen activator inhibitor-1 or lipoprotein(a), antithrombin deficiency, and hyperhomocysteinaemia (3, 4). Although several treatments have been used in LV, mainly including antiplatelet/anticoagulant agents, fibrinolytic/vasodilating medications and anti-inflammatory agent, the condition remains difficult to manage (1, 2). We report here 2 cases of LV associated with hyperhomocysteinaemia, which responded dramatically to folic acid and vitamin B6/B12 supplementation despite being refractory to several other therapies.