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Fig. 5 | Orphanet Journal of Rare Diseases

Fig. 5

From: Linear scleroderma “en coup de sabre” with extensive brain involvement—Clinicopathologic correlations and response to anti-Interleukin-6 therapy

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)

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