Sensory ganglionopathy with livedoid vasculopathy controlled by immunotherapy

Muscle Nerve. 2015 Feb;51(2):296-301. doi: 10.1002/mus.24452. Epub 2014 Dec 23.

Abstract

Introduction: Livedoid vasculopathy is a rare dermatological condition characterized by painful ulceration, atrophic scarring, and persistent livedo reticularis. The pathogenesis is unclear.

Methods: We report a patient with biopsy-proven livedoid vasculopathy who developed a progressive sensory ganglionopathy with profound sensory ataxia. Serial nerve conduction assessments were undertaken.

Results: Combined treatment with prednisolone and mycophenolate mofetil failed to control the ganglionopathy. After addition of rituximab, both symptoms and nerve conduction studies showed stabilization.

Conclusions: Sensory ganglionopathies associated with autoimmune and inflammatory conditions may be characterized by a sub-population of "sick" dorsal root ganglia that can be rescued with aggressive immunotherapy.

Keywords: immunosuppression; livedoid vasculopathy; nerve conduction studies; neuronopathy; sensory ganglionopathy.

Publication types

  • Case Reports

MeSH terms

  • Action Potentials / physiology
  • Adult
  • Ataxia / complications
  • Ataxia / therapy
  • Female
  • Ganglia, Sensory / pathology*
  • Hand / pathology
  • Hand / physiopathology
  • Humans
  • Immunotherapy / methods*
  • Livedo Reticularis / complications*
  • Livedo Reticularis / therapy*
  • Neural Conduction / physiology
  • Peripheral Nervous System Diseases / complications*
  • Peripheral Nervous System Diseases / therapy*
  • Skin / pathology
  • Skin / physiopathology