Skin denervation and cutaneous vasculitis in eosinophilia-associated neuropathy.
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.


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


METHODS
Case series.


METHODS
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/microL).


METHODS
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.


METHODS
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.


CONCLUSIONS
Skin denervation with cutaneous vasculitis is a major manifestation of eosinophilia-associated neuropathy.
Chao
Chi-Chao
CC
Department of Neurology, National Taiwan University Hospital, 7 Chung-Shan S Rd, Taipei 10002, Taiwan.
Hsieh
Sung-Tsang
ST
Shun
Chia-Tung
CT
Hsieh
Song-Chou
SC
D000328:Adult; D000368:Aged; D015415:Biomarkers / Q000032:analysis / Q000378:metabolism; D001706:Biopsy; D015267:Churg-Strauss Syndrome / Q000276:immunology / Q000473:pathology*; D004185:Disability Evaluation; D004568:Electrodiagnosis; D004802:Eosinophilia / Q000150:complications* / Q000276:immunology / Q000473:pathology; D004804:Eosinophils / Q000276:immunology / Q000473:pathology; D005260:Female; D006801:Humans; D007958:Leukocyte Count; D008297:Male; D008833:Microcirculation / Q000276:immunology / Q000473:pathology / Q000503:physiopathology; D008875:Middle Aged; D009069:Movement Disorders / Q000175:diagnosis / Q000276:immunology / Q000503:physiopathology; D009431:Neural Conduction; D009437:Neuralgia / Q000276:immunology / Q000503:physiopathology; D010523:Peripheral Nervous System Diseases / Q000276:immunology / Q000473:pathology*; D011984:Sensory Receptor Cells / Q000276:immunology / Q000473:pathology*; D012867:Skin / Q000098:blood supply / Q000294:innervation / Q000473:pathology*; D043222:Ubiquitin Thiolesterase / Q000032:analysis / Q000378:metabolism; D014657:Vasculitis / Q000276:immunology / Q000473:pathology*
D015415:Biomarkers; C482860:UCHL1 protein, human; D043222:Ubiquitin Thiolesterase