Atypical presentation of livedo racemosa in a factor V Leiden heterozygous positive patient with Pseudomonas aeruginosa urosepsis

Abstract

Impairment of the protein C pathway, detectable by reduced plasma levels of activated protein C (APC), are risk factors for venous thrombosis. Activated protein C maintains clotting homeostasis by regulation of pro-coagulant factors Va and VIIIa. Both infection and the factor V Leiden mutation reduce the formation of APC from protein C in the blood. With low levels of APC, excess factors Va and VIIIa exist, increasing the risk of thrombus formation. Livedo racemosa is characterised by a striking, violaceous branch-like pattering of the skin. It is similar to livedo reticularis, but with a different morphology and histopathology. In this case report we present the first case of livedo racemosa, in an 89-year-old factor V Leiden-positive patient with a Pseudomonas aeruginosa urinary tract infection. The cutaneous biopsies demonstrated vasculopathy with intraluminal thrombi in subcutaneous vessels with no evidence of inflammatory vasculitis.