Muscle Nerve 2014 Dec 23
Sensory ganglionopathy with livedoid vasculopathy controlled by immunotherapy.
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.
