Lee J.-E.CHIA-TUNG SHUNSONG-CHOU HSIEHSUNG-TSANG HSIEH2021-03-182021-03-1820050003-9942https://scholars.lib.ntu.edu.tw/handle/123456789/553259Background: Skin denervation in vasculitic neuropathy has rarely been documented despite frequent manifestations of small-fiber neuropathy including reduced sensitivity and neuropathic pain. Recently, skin biopsy has been established as a new approach to diagnose small-fiber sensory neuropathy. Objectives: To investigate the pathologic features of cutaneous nerves and to evaluate inflammatory vasculopathy in the skin of patients with vasculitis. Design: Case series. Setting: National Taiwan University Hospital, Taipei. Patients: Six patients with vasculitic neuropathy. Interventions: Patients had 3-mm punch biopsy specimens taken from the distal part of the leg (without active vasculitic lesions) and a sural nerve biopsy specimen was taken in addition to detailed neurologic examinations, laboratory investigations, and nerve conduction studies. Main Outcome Measures: Results of nerve conduction studies, epidermal nerve fiber density studies, and immunohistochemistry. Results: All 6 patients had combined large-and small-nervef iber involvement on the neurologic examinations. Nerve conduction studies showed a pattern of axonal neuropathy or mononeuropathy multiplex. Epidermal nerve fiber densities were significantly reduced in the skin of all patients, consistent with concomitant small-fiber neuropathies. Perivascular infiltration by T cells and macrophages was demonstrated by immunohistochemistry. All patients experienced neurologic improvement in muscle strength and alleviation of sensory symptoms after immunotherapy with corticosteroids, plasma exchange, or cyclophosphamide. Conclusions: Small-diameter sensory nerves are affected in vasculitis in addition to the well-known effect of vasculitis on large-diameter nerves. Significant inflammatory vasculopathy is present in the skin despite the absence of clinically active vasculitic lesions. ? 2005 American Medical Association. All rights reserved.[SDGs]SDG3corticosteroid; cyclophosphamide; adult; article; cell infiltration; clinical article; clinical feature; denervation; density; female; human; immunohistochemistry; immunotherapy; inflammation; laboratory; macrophage; male; muscle strength; nerve biopsy; nerve conduction; nerve fiber; neurologic examination; neuropathy; pathology; plasmapheresis; priority journal; punch biopsy; sensory nerve; skin; skin nerve; sural nerve; T lymphocyte; vascular disease; vasculitis; Adolescent; Denervation; Female; Humans; Immunohistochemistry; Male; Middle Aged; Neural Conduction; Peripheral Nervous System Diseases; Skin; Sural Nerve; Vasculitisjournal article10.1001/archneur.62.10.1570162169402-s2.0-26444544500