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Arterial dissection-lentiginosis syndrome

Synonym(s) -
Prevalence <1 / 1 000 000
Inheritance Unknown
Age of onset Childhood
  • Q87.8
MeSH -
MedDRA -


Disease definition

Arterial dissection-lentiginosis is a rare association syndrome, reported in several members of two families to date, and characterized by arterial dissection, occurring at an early age and presenting with a range of manifestations depending on the vascular territory involved (ex. headache, dysphasia, hemiparesis), in association with cystic medial necrosis and multiple lentigines (brown and black in color and mainly affecting the skin of the trunk and extremities).

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Detailed information

Clinical practice guidelines
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