Uncoupling of protein C and antithrombin III activity in cerebral ischemia patients associated with cutis marmorata.

PURPOSE Cutis marmorata is a cutaneous livedoid disorder which can be differentiated from livedo reticularis in both clinical and pathological presentations. Unlike Sneddon syndrome, a detailed immunocoagulation profile has not yet been delineated for cutis marmorata in patients with cerebral ischemia. METHODS To analyze the immunocoagulation profile in cutis marmorata patients associated with cerebral ischemia (CMCI) in a series of 135 cerebral ischemia patients. RESULTS A total of 32 patients were found to have cutis marmorata. The blood protein C activity, protein S activity, antithrombin III activity, platelet count, fibrinogen and frequency of abnormal antiphospholipid antibody level were similar among 32 CMCI patients, 103 cerebral ischemia patients without cutis marmorata, and 35 healthy subjects. However, uncoupling of protein C and anti-thrombin III was observed in CMCI patients. Serum antinuclear antibody and Venereal Disease Research Laboratory were not detected in these patients. CONCLUSION Cutis marmorata is not uncommon in our ischemic stroke patient population, and is characterized by uncoupling of protein C and antithrombin III with altered thrombin hemostasis. Our findings raise the need for a careful cutaneous examination in patients with ischemic stroke. Abnormal immunocoagulating profile should alert physicians to the risk for cerebral ischemia even in the absence of other cardiovascular risk factors.