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

Keywords

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

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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