Cerebrovascular lesions and livedo reticularis (Sneddon's syndrome) ? a progressive cerebrovascular disorder?

Abstract

Four cases are described in which livedo reticularis was associated with repeated cerebrovascular accidents, which eventually resulted in severe disability in two cases. Patients with severe disability had a history of many years, whereas two patients with little or moderate residual disability had a follow-up of 3 years each. CT scan revealed multifocal cerebral infarctions and cortical atrophy in all cases. Repeated cerebral angiograms, done in three cases, showed no signs of a vascular disease. There were no parameters that pointed to active immunological or inflammatory disorder. Neither clinical evidence of heart or large vessel disease was found. Observations suggest that a so-far unknown progressive cerebral vessel disease associated with livedo is the cause of a steady increase in multiple small cerebral infarctions. Because of the progressive character of the disease the search for effective therapy is needed.

Protein Z Deficiency in Antiphospholipid-Negative Sneddon’s Syndrome

“…In any case, treatment rationale relies on the observation of microarterial thromboses in the affected tissues and the assumption that SNS is part of the spectrum of vascular occlusive disorders. Corticosteroids and immunosuppressive agents are notably ineffective in SNS, 2,15,16 and current therapeutic strategy relies on antiplatelet or anticoagulant therapy to prevent recurrence of vascular occlusive events. Although optimal management of SNS patients is yet to be determined through controlled trials, antiplatelet therapy represents a currently reasonable initial approach in aPL-negative patients, whereas preliminary data seem to indicate that anticoagulation is probably more effective than antiplatelet therapy for secondary prevention in the aPL-positive subset.…”

The Mystery of Sneddon Syndrome: Relationship with Antiphospholipid Syndrome and Systemic Lupus Erythematosus

“…However, in some cases, it appears secondly [4,7], or becomes more obvious at the acute phase of cerebral events [9]. Other dermatological manifestations include acrocyanosis, Raynaud's phenomenon and less frequently circumscribed skin necrosis.…”

Sneddon's Syndrome

“…The use of platelet-inhibiting agents like acetylsalicylic acid or dipyridamole may have a modest effect. 16,20,29,30,40 Prophylaxis Obviously, treatment with oral contraceptives should be avoided; the same is true for the other risk factors such as smoking or obesity, even though no ob¬ vious impact was demonstrated in our series. Patients must be warned that pregnancies may bear an in¬ creased risk of deterioration.…”