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Sensory ganglionopathy with livedoid vasculopathy controlled by immunotherapy
James J P Alix1, Marios Hadjivassiliou, Rokiahmah Ali
1Department of Clinical Neurophysiology, Royal Hallamshire Hospital, Sheffield Teaching Hospitals NHS Foundation Trust, Glossop Road, Sheffield, S10 2JF.
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.
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