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Arterial dissection - lentiginosis

ORPHA1682
Synonym(s) -
Prevalence <1 / 1 000 000
Inheritance Unknown
Age of onset Childhood
ICD-10
  • Q87.8
OMIM
UMLS -
MeSH -
MedDRA -

Summary

Arterial dissection-lentiginosis is a rare association syndrome. It has been described in several members of two families. The arterial dissection was characterized by spontaneous cervical-artery dissections at an early age in four of the cases and by aortic dissection in one case (a 22 year-old patient). The etiology of this association is unknown, nevertheless, the embryologic origin of the involved tissues suggests that a neural crest defect may underlie the abnormality in these families. The mode of inheritance is unknown.


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