Widespread cutaneous necrosis occurring in association with the antiphospholipid syndrome: a report of two cases

The antiphospholipid syndrome is an acquired prothrombotic state where thro mbosis and/or pregnancy loss is related to the presence of antiphospholipid antibodies. Cutaneous necrosis secondary to intravascular thrombosis of sm all dermal vessels is a recognized but rare association with ant antiphosph olipid syndrome. We report two patients with high circulating levels of ant icardiolipin antibodies who developed widespread cutaneous necrosis as the first clinical manifestation of the antiphospholipid syndrome. The exact me chanism by which antiphospholipid antibodies mediate thrombosis is uncertai n; however, proposed mechanisms of activity include endothelial cell activa tion. altered endothelial production of prostacyclin, activation of platele ts, and modulation of the protein C and S pathways.