A 41-year-old man with
hypertension and
hyperlipidemia who complained of left
hemiparesis after a temporal
headache was admitted to our hospital. A cervical MRI with
gadolinium enhancement revealed an intramural
hematoma is compatible with right extracranial
internal carotid artery dissection. Two weeks later, he complained of sudden onset of
pain in the right side of his neck. The right extracranial
internal carotid artery dissection followed by the right extracranial
vertebral artery dissection was diagnosed. Spontaneous cervical
artery dissection (SCAD) is one of the causes of
stroke in young adults. The pathogenesis of SCAD remains unknown. Minor
trauma like an excessive sneeze,
migraine, and connective tissue disorders such as
fibromuscular dysplasia and
Ehlers-Danlos syndrome are well-known as risk factors for SCAD. Pathologically skin
collagen abnormalities have been seen in German patients with SCAD without clinical evidence for any specific connective tissue disorder. We examined the ultrastructural morphology of the Japanese patient's dermal connective tissue components by electron microscopy. The patient's
collagen fibers contained fibrils with highly variable diameters, and there were other ultrastructural abnormalities, including flower-like fibrils and large-diameter composite fibrils. This is the first report of a case of ultrastructural abnormalities of dermal connective tissue in a Japanese patient with SCAD.