Livedo Reticularis and Cerebrovascular Lesions (Sneddon's Syndrome)

Abstract

Eight patients with diffuse livedo reticularis and cerebrovascular lesions (Sneddon's syndrome) are reported. The disorder was inherited by autosomal dominant transmission in 3 cases. Multiple occlusions in medium-sized arteries were demonstrated by cerebral and hand arteriograms. Digital artery biopsies showed intimal hyperplasia in 7 cases and recanalized thrombosis in one case. Our findings are compared with an extensive review of the literature. Differential diagnosis with other vascular disorders, especially cerebral thromboangiitis obliterans and the corticomeningeal angiomatosis of Divry and Van Bogaert is considered. We conclude that Sneddon's syndrome is a new genetic and progressive arteriopathy, occlusive and noninflammatory, involving medium-sized vessels. The pathogenesis has yet to be elucidated.

Sneddon's syndrome with antiphospholipid antibodies and arteriopathy.

“…While cerebral angiography in patients with cerebrovascular disease and antiphospholipid antibody syndrome has either been normal or revealed large-vessel occlusions, 2 middle-sized arteries are involved in Sneddon's syndrome. 3 ' 4 However, we did not suggest that the pathogenesis of cerebral ischemia in Sneddon's syndrome was different whether antiphospholipid antibodies were present or not.…”

“…The experiments were performed on 2 consecutive days, and the daily schedule was exactly the same as that described previously. 1 At 12 midnight on the second experimental day, the patients were given 250 ml of an electrolytic drink containing (meq/1) Na + 21, K + 7, Ca 2+ 1, Mg 2 * 0.5, C\' 18.5, citrate 3 " 10, and lactate" 1 (POCARI SWEAT, Otsuka Pharmaceutic Co., Tokyo). Hematocrit and blood viscosity were determined at 4-hour intervals from 8 AM one day until 8 AM the following day.…”

“…3 Its pathogenesis is unknown, but there are some reports of Sneddon's syndrome associated with aPL antibodies, 4 -6 suggesting that these antibodies may play a role. Nowadays, it seems clear that the association of livedo reticularis with cerebrovascular lesions could be the result of a wide spectrum of causes, one of which is the "primary" antiphospholipid syndrome.…”

Cerebral Infarction in Young Women: Analysis of 130 Cases

“…The etiology of cerebral infarction was classified as follows: (1) atherosclerosis, evidence of vascular risk factors and atheromatous lesions on Doppler and/or cerebral angiography related to clinical manifestations, (2) cardioembolism, presence of cardiac conditions associated with embolism with echocardiographic confirmation of embolic source [15]; the presence of patent foramen ovale (PFO) or MVP with myxomatous degeneration with no other potential cause of stroke was considered as a cause of cardioembolic cerebral infarction, (3) nonatherosclerotic vasculopathy, including spontaneous cervicocerebral arterial dissection [16], fibromuscular dysplasia [17], Takayasu's arteritis [18], parasitic arteritis (cysticercosis) [19], primary central nervous system arteritis [20], and drug-induced vasculopathy [21], (4) hematologic factors, including natural anticoagulant factor deficiencies [22], antiphospholipid antibodies (only considered as etiologic evidence in the absence of another cause of cerebral infarction) [23], hypercoagulable state related to pregnancy and puerperium [24], Sneddon's syndrome [25], and resistance to activated C protein [26], (5) migraine [14], and (6) cryptogenic factors, when based on the above criteria it was not possible to determine the etiology.…”

Study of antiphospholipid antibodies in a patient with Sneddon's syndrome and her family.

“…2 The etiopathogenesis of this disorder is still unknown. A number of reports have documented a link between antiphospholipid antibodies (aPLs), including lupus anticoagulant (LA) and anticardiolipin antibodies (aCLs), and thrombotic events with different results.…”