Tissue vulnerability revealed by ultrastructural skin analysis as a surrogate for arterial integrity in two cases of vascular Ehlers-Danlos syndrome

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Clinical presentation and operative outcomes in vascular Ehlers-Danlos syndrome (VEDS) are heterogeneous. We present two patients with genetically confirmed VEDS who exhibited markedly different iliac pathology phenotypes. One ruptured a common iliac artery at a near-normal diameter and the other tolerated a large aortoiliac aneurysm without rupture. The Aortic and Arterial Vulnerability Spectrum is a conceptual framework that characterizes aortic and arterial failure (dissection/rupture) along a continuum of underlying tissue vulnerability, integrating extracellular matrix (ECM) ultrastructure and clinical phenotypes beyond traditional diameter-based models. Using ultrastructural analysis of skin biopsy as a window into arterial integrity, we demonstrate distinct differences in ECM organization that paralleled the vascular phenotypes. Viewed through the Aortic and Arterial Vulnerability Spectrum, these cases show how genotype-driven vulnerability shapes surgical phenotype in VEDS and highlight ultrastructural skin analysis as a tool for ECM assessment and possibly biologic risk stratification.

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