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Livedoid vasculopathy: A challenging disease to diagnose

Livedoid vasculopathy (LV) (often mistaken for vasculitis) is a non-inflammatory occlusion of the small vessels of the skin due to thrombosis and deposition of fibrin in the vessel walls. The disease affects primarily the small blood vessels in the lower extremities and is characterised by persistent livedo racemosa associated with recurrent painful ulcerations. The aetiology of LV has not been completely elucidated, but the disease is considered an occlusive thrombotic process due to a hypercoagulable state. Herein, we present the case of an 11-year-old child with no acquired thrombophilic risk factors who was diagnosed with LV. A detailed thrombophilia workup demonstrated homozygosity for the plasminogen activator inhibitor-1 (PAI-1) 4G allele (4G/4G genotype). This case is presented to emphasise the importance of investigating prothrombotic conditions in LV in the differential diagnosis of vasculitis in patients with painful leg ulcers.