Abstract:
:Moyamoya angiopathy (MA) is a progressive cerebrovascular disease with a poorly understood pathophysiology. It is mainly characterized by progressive bilateral stenosis of the terminal intracranial part of the supraclinoid internal carotid arteries and the proximal parts of the middle and anterior cerebral arteries, as well as the development of a fragile collateral network at the base of the brain. This results in early-onset ischemic or hemorrhagic strokes. The disease may be idiopathic (known as Moyamoya disease) or associated with other heritable or acquired conditions, including type 1 neurofibromatosis or other RASopathies, sickle cell disease, Down syndrome, or autoimmune disorders (known as Moyamoya syndrome). Although the disease is thought to selectively involve the brain vessels, systemic manifestations such as cardiac and ocular ones have been reported in MA, mostly in the syndromic form. Various cutaneous manifestations have also been described in MA patients. These include changes in the epidermis, dermis, or skin appendages for example café-au-lait spots, hypomelanosis of Ito, livedo racemosa, hemangiomas, premature graying of hair, etc. The present review summarizes the cutaneous associations as well as the coincidental dermatological findings seen in MA patients.
journal_name
Eur J Neuroljournal_title
European journal of neurologyauthors
Mitri F,Bersano A,Hervé D,Kraemer Mdoi
10.1111/ene.14754subject
Has Abstractpub_date
2021-01-23 00:00:00eissn
1351-5101issn
1468-1331pub_type
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