Summary
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.
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Rumpl, E., Neuhofer, J., Pallua, A. et al. Cerebrovascular lesions and livedo reticularis (Sneddon's syndrome) — a progressive cerebrovascular disorder?. J Neurol 231, 324–330 (1985). https://doi.org/10.1007/BF00313710
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DOI: https://doi.org/10.1007/BF00313710