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Chi-Chao Chao, MD; Sung-Tsang Hsieh, MD, PhD; Chia-Tung Shun, MD; Song-Chou Hsieh, MD, PhD

Arch Neurol. 2007;64(7):959-965.

Background  Eosinophilia is frequently associated with peripheral neuropathy, and neuropathic pain is a major presentation. Little is known about the involvement of sensory nerve terminals and the vasculature in the skin of patients with eosinophilia.

Objectives  To investigate the skin innervation and the pathological abnormalities of the cutaneous vasculature and their clinical significance in eosinophilia-associated neuropathy.

Design  Case series.

Setting  National Taiwan University Hospital, Taipei, Taiwan.

Patients  Twelve patients with neuropathy and concomitant eosinophilia (with an eosinophilic ratio of white blood cell classification > 10% or absolute eosinophil count of > 1000/µL).

Interventions  Clinical assessments of neurological deficits, laboratory tests, nerve conduction studies, and a skin biopsy specimen 3 mm in diameter taken from the distal leg without active skin lesions.

Main Outcome Measures  Quantitation of epidermal innervation, immunopathological findings of the cutaneous vasculature, and motor disability grade.

Results  Six patients fulfilled the criteria of Churg-Strauss syndrome, and the other 6 patients were categorized as having primary eosinophilia. All of the 12 patients had mononeuropathy multiplex or polyneuropathy with sensory symptoms as the initial manifestation. Intraepidermal nerve fiber densities were reduced in 10 patients (83.3%), being significantly lower than in the controls (mean ± SD, 2.12 ± 2.30 vs 10.56 ± 3.69 fibers/mm, respectively; P < .001) and negatively correlated with the disability grade (P = .003). Nine patients (75.0%), including all of the 6 patients with Churg-Strauss syndrome, had cutaneous vasculitis, and two-thirds of the 9 patients had perivascular infiltration of eosinophils.

Conclusion  Skin denervation with cutaneous vasculitis is a major manifestation of eosinophilia-associated neuropathy.

Author Affiliations: Departments of Neurology (Drs Chao and S.-T. Hsieh), Pathology (Dr Shun), and Internal Medicine (Dr S.-C. Hsieh), National Taiwan University Hospital, and Departments of Anatomy and Cell Biology (Dr S.-T. Hsieh) and Forensic Medicine (Dr Shun), National Taiwan University College of Medicine, Taipei, Taiwan.

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