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Livedoid microangiopathy associated to antiphospholipid antibody syndrome (APS)

Livedoid microangiopathy is a chronic segmental hyalinizing syndrome of dermal blood vessels, of unknown origin, that affects mainly the lower limbs. Histopathologically, both initial as well as late lesion are characterized by few inflammatory cells infiltrate, with prevalent capillary angiogenesis and microthrombi, agglomerate of small capillaries, and dermal deposits of hemosiderin. The absence of a substantial perivascular infiltrate or leukocytoclasia argues against vasculitis, favouring a thrombotic process. Livedoid microangiopathy attacks mainly young and middle-aged women; can be idiopathic, or associated with coagulation alterations including the factor V Leiden mutation, protein C deficiency, increased plasmatic homocysteine, fibrinolysis abnormalities, platelet activation and antiphospholipid antibody syndrome (APS). We describe a case of a patient with livedoid microangiopathy associated with the presence of APS with multiple ulcers in the lower limbs who showed a clinical improvement only after total anticoagulation with warfarin and association with danazol. Livedoid vasculitis can represent an initial clinical manifestation of a group of diseases which cause occlusive vasculopathy; so, every patient should be investigated for the presence of antiphospholipid antibody or of another cause of thrombophilia.

livedoid microangiopathy; antiphospholipid syndrome; thrombophilia


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