"Small-Vessel Vasculitis or Perifolliculitis in Small-Fiber Neuropathy " by Lawrence A. Zeidman, Todd Levine et al.
 

Small-Vessel Vasculitis or Perifolliculitis in Small-Fiber Neuropathy With TS-HDS, FGFR-3, or Plexin D1 Antibodies

Document Type

Article

Publication Date

12-1-2024

Publication Title

J Clin Neuromuscul Dis

Abstract

INTRODUCTION: Small-fiber neuropathy (SFN) is highly prevalent but often idiopathic. TS-HDS, FGFR-3, and Plexin D1 autoantibodies (seropositive) may be present in more than 40% of idiopathic cases. Another autoimmune biomarker is a non-length-dependent (NLD) skin biopsy pattern. Our goal was to demonstrate that small-vessel vasculitis and perifolliculitis (inflammation) on skin biopsies are additional biomarkers.

METHODS: All pure SFN skin biopsy reports were reviewed for inflammation, and their charts were examined for other relevant history.

RESULTS: Seven of 80 patients with pure SFN had inflammation (8.8%); 5 patients were female (71%) and 2 were male (29%); average age was 45 (16-67). All 7 patients with inflammation were seropositive (100%, P = 0.0495), and 6 patients (86%) had either NLD inflammation or NLD pathology (P = 0.0003).

DISCUSSION: Inflammation is present only in a small portion of punch biopsies, but may be another autoimmune SFN biomarker. It is strongly associated with seropositivity and NLD-pathology. Further studies are likely indicated to assess inflammation pathophysiology and immunotherapy responsiveness.

Medical Subject Headings

Humans; Male; Female; Middle Aged; Adult; Small Fiber Neuropathy; Aged; Vasculitis; Autoantibodies; Young Adult; Adolescent; Skin; Nerve Tissue Proteins; Receptors, Cell Surface; Biopsy; Membrane Glycoproteins; Intracellular Signaling Peptides and Proteins

PubMed ID

39590924

Volume

26

Issue

2

First Page

63

Last Page

69

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