By Gary M. White, MD
Livedoid vasculopathy 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.
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.
Workup should exclude lupus, cryoglobulinemia, antiphospholipid syndrome, vasculitis (e.g., cutaneous polyarteritis nodosa), factor V Leiden heterozygous mutation, and chronic venous insufficiency. Interventions may include biopsy, ANA, antiphospholipid antibody, lupus anticoagulant, factor V Leiden, cryoglobulins, skin biopsy, and venous Doppler studies. In one series, 58% of patients had positive antiphospholipid antibodies [Acta Derm Venereol. 2013 Dec 17].
Treatment is challenging. Workup for systemic conditions associated with hypercoagulability or vaso-occlusion should be performed. Smoking cessation is critical. Leg elevation and compression therapy are important adjuncts.
Rivaroxaban (10-20 mg daily), an oral factor Xa inhibitor, has led to rapid improvement in both pain and the size of ulcers in several patients [BJD 2015;172;1148 and BJD 2013;168:898]. No laboratory monitoring is required, but hemorrhage is a risk.
Monshi et al reported excellent results with IVIG in a study of 11 patients [JAAD 2014;71;738]. They utilized a high dose, 2 g/kg body weight over 2-3 consecutive days every 4 weeks for 6 months. Many achieved complete remission after 3 cycles. Resolution of pain was even sooner, e.g., after 1 or 2 cycles.
Clopidogrel disulfate (Plavix) 75 mg/day has been recommended anecdotally. Other treatment options include other antiplatelet agents (aspirin or dipyridamole), vasodilating agents (nifedipine), agents that alter blood viscosity and erythrocyte plasticity (pentoxifylline), fibrinolytic agents (danazol or tissue plasminogen activator), anticoagulant agents (heparin or warfarin), and immunomodulating agents (hydroxychloroquine or immunosuppressants).
In some patients, combination therapy may be considered to maximize benefit.
After the very painful ulcer heals, it usually leaves a white scar.
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