Sneddon‘s syndrome as a disorder of small arteries with endothelial cells proliferation: ultrastructural and neuroimaging study
Journal Title: Folia Neuropathologica - Year 2005, Vol 43, Issue 4
Abstract
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 CD31, CD34, 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.
Authors and Affiliations
Eliza Lewandowska, Teresa Wierzba-Bobrowicz, Romana Bogusławska, Anna Rudnicka, Elżbieta Pasennik, Waldemar Lechowicz, Tomasz Stępień, Włodzimierz Kuran
Keywords
Related Articles
- EP ID EP123517
- DOI -
- Views 74
- Downloads 0
Teratoma or enterogenous cyst? The histopathological and clinical dilemma in co-existing occult neural tube dysraphism
Background: Better understanding of embryology, histopathology and genetics of dysraphic conditions have lead to an expansion of this concept to entities with a similar microscopic appearance (e.g. enterogenous cysts, co...
The interaction between lung cancer metastases to the brain and their surroundings
Background and study purpose: due to the fact that an interrelation between metastases of lung cancer to the brain and surroundings may influence the prognosis, we made attempts to answer the following questions: 1) how...
Materials from XIII Conference of Polish Association of Neuropathologists Warszawa, May 12-14, 2005 Original article The diagnosis and therapy of brain tumours
Neoplasms arising from glial cells make up the most common group of primary brain tumors. The clinical outcome, especially the survival rates of the patients with brain tumours depend on tumour grade expressing its mal...
Hemosiderin pigmentation of tumour cells in cerebellar pilocytic astrocytoma associated with post-traumatic hemorrhage in adults
The pilocytic astrocytoma is only rarely associated with gross intratumoral hemorrhage despite rich vasculature and blood vessel changes, accompanied often by perivascular depots of hemosiderin. We report an unusual cas...
Autophagy – a basic mechanism and a potential role for neurodegeneration
Autophagy constitutes a fundamental survival strategy of cells; its disturbance contributes to the pathogenesis of cancer, liver and immune disease, pathogen infection, myopathies as well as neurodegenerative disorders s...