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. 2019 May 16;14:110. doi: 10.1186/s13023-019-1015-7

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© The Author(s). 2019

Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

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Fig. 5 — Neuropathological Findings: Direct and Indirect Immunofluorescent Studies. a There is deposition of IgG within the blood vessels showing direct endothelial cell localization indicative of antibodies of IgG isotype targeting the endothelium (× 100) (+ 3/3 staining intensity) b IF Studies showing striking reactivity for C5b-9 in blood vessels; C5b-9 is the effector mechanism of autoimmune endothelial cell injury syndromes including dermatomyositis, Kohlmeier-Degos disease in the setting of collagen vascular disease and Susacs syndrome (× 100)(+ 3/3 staining intensity). c The indirect immunofluorescent studies to assess for antiendothelial cell antibodies showed striking nuclear reactivity of generic endothelial cells with the patient’s serum. The pattern of reactivity suggests reactivity of generic endothelial cells with a para anti-centromere pattern using conventional Hep-2 substrates, (× 100) (+ 3/3 staining intensity). d Patient serum was incubated with her brain and showed the same pattern of reactivity as by DIF. Background cells are positive, showing a distinct scleroderma-like pattern (× 100) (+ 3/3 staining intensity)