Degos' disease: association with anticardiolipin antibodies and the lupus anticoagulant.

Antiphospholipid thrombosis syndromes

“…Because of a noted association among lupus, a biologic false-positive test result for syphilis, and the presence of the LA, in 1983 Harris et al [57] devised a new test for antiphospholipids using cardiolipin. This test and subsequent modifications now have become known as the anticardiolipin antibody test; generally, IgG, IgA, and IgM anticardiolipin idiotypes currently are assessed [58]. Shortly after development of the ACLA assay, it became apparent that these antibodies were not limited to the population of patients with lupus but were found in non-lupus patients as well.…”

Lupus Anticoagulant and Lacunar Infarctions

“…Most re ported patients have not had identifiable cardiac lesions threatening cerebral embolization or occlusive carotid disease potentially relieved by endarterectomy. One of two patients with TIA [7] had recurrence of cerebral ischemia on antiplatelet therapy but responded to anti coagulation, as did the patients with strokes reported by Elias and Eldor [6] and Kelley et al [9], Our patient with lupus anticoagulant but no evidence of lupus responded well to antiplatelet agents, while the patient who had lupus responded well to steroid therapy, as did the patient of Englert et al [8] with strokes and Degos' dis ease. Our cases suggest that infarctions, lacunar or other wise, associated with lupus anticoagulant are best treated according to the presence or absence of underlying sys temic disorder.…”

“…Recurrent TIA involving var ious arterial territories in young adults who had no angiographic evidence of occlusive cerebrovascular dis ease were reported by Landi et al [7], while multiple strokes accompanied lupus anticoagulant in a young woman with Degos' disease described by Englert et al [8]. Kelley et al [9] described cerebral infarctions in two middle-aged men who had lupus anticoagulant without apparent underlying medical illness, but who had risk factors for cerebrovascular disease.…”

“…Despite this prolongation of bleeding indices, hemorrhagic complica tions are infrequent [4], A tendency to thromboembo lism has instead been encountered [5,6], Cerebral thrombotic events have been increasingly recognized [7][8][9]. Transient ischemic attacks (TIA) [7] and large-vessel thrombotic infarctions [8][9][10] have been described, but lacunar infarctions have not heretofore been reported. Lacunes were initially reported in hypertensive patients [11], and an increasing number of syndromes have been recognized in atherosclerotic as well as hypertensive cerebrovascular disease [12], We have observed two cases of demonstrable lacunar infarction associated with lupus anticoagulant.…”

“…It was first described in patients with systemic lupus ery thematosus (SLE) [2], but has subsequently been associ ated with malignancy and inflammatory disorder, and occurred without any underlying disease [3]. Despite this prolongation of bleeding indices, hemorrhagic complica tions are infrequent [4], A tendency to thromboembo lism has instead been encountered [5,6], Cerebral thrombotic events have been increasingly recognized [7][8][9]. Transient ischemic attacks (TIA) [7] and large-vessel thrombotic infarctions [8-10] have been described, but lacunar infarctions have not heretofore been reported.…”