Unusual Clinical Manifestations of the Antiphospholipid Syndrome

The antiphospholipid syndrome (APS) is characterised by arterial and/or venous thrombosis and pregnancy morbidity in the presence of anticardiolipin antibodies (aCL) and/or lupus anticoagulant (LA). APS can occur either as a primary disorder (PAPS) or secondary to a connective tissue disease, more frequently systemic lupus erythematosus (SLE). Any organ and any size of vessel can be affected in this disorder. In 1983 Hughes, in his original description of the syndrome, also reported thrombocytopenia, neurological disease, livedo reticularis and labile hypertension. The range of clinical features that has been associated with the presence of antiphospholipid antibodies (aPL) is extremely wide and has broadened over the last 20 years.