Skin involvement in antiphospholipid syndrome

Since the first description of antiphospholipid syndrome (APS) [1], a number of dermatological manifestations have been described (Table 12.1) [2–5]. The skin involvement is frequently the presenting feature of the syndrome and, although none of the cutaneous lesions is pathognomonic for APS, some of them may be helpful for its diagnosis [2–5]. The cutaneous manifestations of APS range from mild symptoms like livedo reticularis to life-threatening conditions such as widespread skin necrosis. Livedo reticularis represents the skin sign most frequently seen in APS and is significantly associated with ischemic arterial events [2, 3]; on the other hand, anetoderma, malignant atrophic papulosis, ulcerations resembling pyoderma gangrenosum, and widespread necrosis are conditions regarded as rarely occurring during the course of APS [2].