Chronic leg ulcerations associated with livedoid vasculopathy successfully treated with rivaroxaban

Livedoid vasculopathy (LV) is a prothrombotic chronic skin disease caused by the recurrent occlusion of dermal microcapillaries and subsequent ischaemia. LV presents with painful, recurrent and recalcitrant ulcerations of the legs, which result in irreversible scarring. It mostly affects the malleolar region and dorsal surface of the feet. In a recent analysis of the German diagnosis-related group data for LV, the male/female ratio was 2.1 : 1 and patients aged 45 years comprised 74.7% of all patients (with peaks at the ages of 45–50 and 70–75 years). The aetiology of LV has not been completely elucidated, but it may be associated with various coagulopathies and associated diseases, such as antiphospholipid syndrome, lupus erythematosus, dysproteinaemias, genetic diseases such as as factor V Leiden and prothrombin G20210A gene mutations, reduced protein C or protein S activity, low levels of endogenous tissue plasminogen activator, increased levels of plasminogen activator inhibitor and prothrombotic markers such as homocysteine or lipoprotein, and other disorders associated with abnormal platelet activation or fibrinolysis. LV is often misdiagnosed and may be underdiagnosed. Treatment is usually challenging, with the main goals being pain relief and stopping disease progression. Therapeutic options include subcutaneous low molecular weight heparin, warfarin, aspirin, dipyridamole, pentoxifylline, nifedipine, sulfasalazine and intravenous immunoglobulin, among others. Recently, rivaroxaban, a direct factor Xa inhibitor, which can be taken orally and requires no laboratory monitoring, was shown to be effective in the treatment of LV. We report the case of a patient with recalcitrant painful ulcerations and scars who was successfully treated with rivaroxaban after various therapies, including alprostadil, certoparin, enoxaparin, pentoxifylline, prednisolone and methylprednisolone, had failed. The patient was a 32-year-old woman who presented for the first time at our department in 2012. She reported asymptomatic point-shaped redness involving both legs since she was 15 years old. She also had a 1-year history of mottled skin and painful ulcerations on both ankles. The patient was an exsmoker, having stopped about 2 years prior to presentation; she had previously smoked about 14 cigarette packs per year since she was 14 years old. Physical examination revealed mottled skin and ulcerations on both malleolar regions (Fig. 1a), with reticular, flattened, livid erythema on both legs. Punched-out small ulcerations in the ankles with whitish scars, similar to atrophie blanche, were also present. Both lower legs had a symmetrical appearance. Both pedal pulses were palpable and normal. Histological examination of a skin biopsy taken from the left lower leg showed an occluding thrombotic vasculitis of the small and medium vessels consistent with LV (Fig. 2). Except for an elevated homocysteine level (with normal vitamin B6 and B12) and slightly lower folic acid value, laboratory tests were unremarkable, including lipoprotein A, protein electrophoresis, coagulation studies, anticardiolipin antibodies and lupus anticoagulant, factor V Leiden and prothrombin mutations, protein C and S activities, cryoglobulins, antinuclear antibodies and anti-double-stranded DNA antibodies, respectively. Because of the smoking history, folic acid replacement was also initiated. From the time of the patient’s first consultation, she was prescribed a number of therapies, in the following Correspondence: Dr Diana Miguel, Department of Dermatology, University Hospital Erfurter Straße 35, D 07740 Jena, Germany E-mail: diana.miguel@med.uni-jena.de