Clinicopathological Features of Churg-Strauss Syndrome With Severe Nerve Degeneration: A Case Report

Jing Yan Xiang¹, Yu Wu Zhao¹, Jian Liang Fu¹, Xiao Juan Cheng¹, Qin Xue², Yin Wang³

¹Department of Neurology, Sixth People’s Hospital, Shanghai Jiaotong University, ²Department of Nephrology, Sixth People’s Hospital, Shanghai Jiaotong University, ³Department of Neuropathology, Huashan Hospital, Fudan University, Shanghai, China

Key words: Churg-Strauss syndrome; vasculitis; allergic granulomatosis.

Summary. Churg-Strauss syndrome (CSS) is a rare autoimmune vasculitis of unknown etiology that involves small- and medium-sized blood vessels. Its onset is thought to be associated with adult-onset asthma, and vasculitis typically involves vessels in the lungs. However, due to increased blood and tissue eosinophilia, vasculitis may result in the involvement multiple systems of (neurological, skin, etc.). We report a case of CSS with manifestations that included skin purpura and severe peripheral nerve degeneration in a 56-year-old woman with a recent history of asthma. After the treatment with methylprednisolone and standard immunosuppressive therapy, her rashes resolved, there were no acute asthma attacks, and the numbness in her lower limbs improved.

Correspondence to Y. Wu Zhao, Department of Neurology, Sixth People’s Hospital, Shanghai Jiaotong University, 200233 Shanghai, China. E-mail: zhaoyuwu2005@126.com

Received 21 September 2011, accepted 20 May 2012