Correlations between peripheral neuropathy profiles and vascular skin lesions.

BACKGROUND

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.

OBJECTIVE

To determine (1) whether phenotypic correlations exist between neuropathy profiles and vascular skin lesion subtypes, and (2) whether these syndromes share a specific mechanism.

METHODS

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.

RESULTS

Forty-one patients (median age 54 years) were included: 27 had mononeuritis multiplex (MM) and 14 polyneuropathy (PNP). Purpura (p = 0.001) and livedo (p < 0.05) were associated with MM, while ulcers were linked to PNP (p < 0.001). Ulcers were plantar in PNP and supraplantar in MM. Median delay between neuropathy and skin lesion was 1 month in MM versus 58 months in PNP (p < 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 (n = 19), type I cryoglobulinemia (n = 6), and primary antiphospholipid syndrome (n = 2)], whereas PNP was due to diabetes (n = 9), chronic renal failure (n = 1), alcohol (n = 1), leprosy (n = 1), or transthyretin-amyloidosis (n = 2).

DISCUSSION: 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.