The authors report a case of a 7-year-old girl with a progressive sensorimotor peripheral neuropathy with acute relapses associated with a desquamating purpuric rash, alopecia, and spinal cord atrophy. Distinctive features include systemic involvement with alopecia and rash, peripheral nervous system involvement with relapsing and remitting sensorimotor neuropathy, and central nervous system involvement with episodes of dystonic posturing and spinal cord atrophy. Extensive diagnostic evaluation for known causes of skin manifestations associated with peripheral neuropathy has failed to identify a cause for this patient's condition. The authors discuss the case as an approach to the differential diagnosis and to highlight unusual features of this yet to be identified clinical entity.