[Ischemic cerebral vascular accidents in young subjects and livedo reticularis. Apropos of a case of Sneddon's syndrome or Divry-Van Bogaert's syndrome].

The authors report the case of a 28-year-old patient presenting with successive ischaemic cerebral vascular accidents, preceded by the appearance of a livedo reticulare, without any laboratory signs of an inflammatory syndrome. Since Sneddon, and before him Divry and Von Bogaert, patients with this dermatosis are known to have an increased frequency of ischaemic cerebral vascular accidents. This syndrome, considered for a long time to be minimally aggressive, appears to be a very serious disease, leading to a bedridden state and dementia. The prognosis for these patients, all young (30-40 years), therefore appears to be very poor. Any case of livedo reticulare requires the search for cerebral neurological lesions. After a complete neurological examination, imaging therefore has an important major in the assessment of cerebral lesions due to this corticomeningeal angiomatosis. CT scan and MRI are useful in this initial assessment and in the subsequent follow-up of patients. In cases with frank neurological lesions, cerebral arterial exploration is essential with demonstration of lesions of the midcerebral arteries and activation of collateral vascular anastomoses and their helicine vessels. The prognosis is based on the extent of these lesions. Cerebral biopsy may demonstrate a non-inflammatory endothelial cellular proliferation of midcerebral vessels.