In livedoid vasculopathy, a stellate, white scar results upon healing.

LIVEDOID VASCULOPATHY

Livedoid vasculopathy (LV) is a painful, occlusive process of the vessels of the lower legs that starts as painful ulcerations and results in atrophic, white scars. It is also known as atrophie blanche, livedo reticularis with summer ulcerations, segmental hyalinizing vasculitis, PURPLE (painful purpuric ulcers with reticular patterning on the lower extremities) and livedo vasculitis.

• Painful ulcerations of the lower legs in a woman.
• The pathogenesis is occlusion of the cutaneous capillary microcirculation leading to thrombosis, ischemia and infarction. The thrombotic effect results from defects either in the endothelial cell plasminogen activation, platelet dysfunction or enhanced fibrin formation.
• The prothrombotic tendency may be promoted and/or caused by lupus anticoagulant and anticardiolipin antibodies, increased level of plasminogen activator inhibitor, low level of tissue plasminogen activator activity, increased serum homocysteine level, protein C and protein S deficiency, antithrombin III deficiency, prothrombin G20210A gene mutation, factor V Leiden mutation and abnormalities in fibrinolytic pathways.

Clinical

The initial lesion is often a purpuric papule, plaque, or a hemorrhagic bulla that breaks down to form a painful ulcer. Female to male ratio is 3:1. A stellate, white scar results upon healing. A middle-aged woman with chronic venous insufficiency is typical. Peripheral neuropathy may occur.

RegionalDerm

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