Sneddon's syndrome as a disorder of small arteries with endothelial cells proliferation: ultrastructural and neuroimaging study.

[sneddon syndrome]

We report a 18 -year -old female patient with livedo reticularis and neurological disturbances . CT scan showed two big ischemic focuses in the pons , moreover MRI revealed small disseminated ischemic focuses in the pons and deep structures of both brain hemispheres . MRA demonstrated no changes in the big extracranial and intracranial arteries . Since the clinical data and neuroimaging results suggested Sneddon 's syndrome , the skin and skeletal muscle biopsy was taken to examine . The immunohistochemical and ultrastructural investigations of the skin biopsy revealed a significant reduction of the lumen of the capillaries and small to medium-sized arteries . Cells surrounding the vascular lumen , frequently with multilayer arrangement and their nuclei placed perpendicularly to the lumen , were CD 31 , CD 34 , and sporadically SMA positive . At the ultrastructural level , these proliferating cells showed typical features of endothelial cells : abundant intermediate filaments and Weibel-Palade bodies . Between the endothelial cells some junctions were detached as well in the capillaries as in the small arteries . The smooth muscle cells of the small arteries were electron denser than usual and their cytoplasmic protrusions penetrated to the endothelial cells . The ultrastructural picture of some vessels with a considerably narrow lumen was typical of vessels newly formed during angiogenesis . Neuroimaging including TC , MRI , MRA besides histological , immunohistochemical and ultrastructural evaluation may be useful for diagnosis of Sneddon 's syndrome .

Diseases
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Diseases presenting "frequently with multilayer arrangement and their nuclei placed perpendicularly to the lumen" symptom

sneddon syndrome

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