Background <p>Peripheral neuropathies may present with vascular skin lesions, such as livedo racemosa, ulcers, palpable purpura, or necrosis. These rare neurocutaneous associations can be severe and life-threatening.</p> Objective <p>To determine (1) whether phenotypic correlations exist between neuropathy profiles and vascular skin lesion subtypes, and (2) whether these syndromes share a specific mechanism.</p> Methods <p>We conducted a monocentric retrospective study at Montpellier University Hospital (1993–2022) including patients with peripheral neuropathy and vascular skin lesion(s) occurring as a syndrome. Clinical, electrophysiological, and histopathological data were analyzed.</p> Results <p>Forty-one patients (median age 54&#xa0;years) were included: 27 had mononeuritis multiplex (MM) and 14 polyneuropathy (PNP). Purpura (<i>p</i> = 0.001) and livedo (<i>p</i> &lt; 0.05) were associated with MM, while ulcers were linked to PNP (<i>p</i> &lt; 0.001). Ulcers were plantar in PNP and supraplantar in MM. Median delay between neuropathy and skin lesion was 1&#xa0;month in MM versus 58&#xa0;months in PNP (<i>p</i> &lt; 0.001). In PNP subgroup, the neuropathy systematically preceded skin lesion. MM etiologies involved ischemic mechanisms related to vasculitis and/or vaso-occlusion [systemic angiitis (<i>n</i> = 19), type I cryoglobulinemia (<i>n</i> = 6), and primary antiphospholipid syndrome (<i>n</i> = 2)], whereas PNP was due to diabetes (<i>n</i> = 9), chronic renal failure (<i>n</i> = 1), alcohol (<i>n</i> = 1), leprosy (<i>n</i> = 1), or transthyretin-amyloidosis (<i>n</i> = 2).</p> Discussion <p>MM correlates with livedo, purpura, and supraplantar ulcers, reflecting ischemia from vasculitis or vaso-occlusion. Conversely, plantar ulcers result from chronic PNP affecting large and small fibers. Skin lesion type may guide neuropathy phenotype and underlying etiology.</p>