患者 ,男 ,2 1岁 ,自幼双上、下肢皮肢出现色素沉着 ,随年龄增长皮下出现多发散在小结节病灶 ,近日全身出现多发性神经痛 ,伴四肢乏力 ,活动欠灵活。CT检查 :颈、腰椎管内见不规则略低密度影 ,向双侧神经孔方向延伸 ,CT值约 6 6 Hu～ 77Hu,密度均匀 ,双侧椎体附件破坏 (见图 ) Patients, male, 21 years old, since childhood, both upper and lower limbs appear pigmented skin, with age, subcutaneous appear more divergent in small nodules lesions, recent systemic multiple neuropathic pain, accompanied by weakness, activity inflexible. CT examination: The neck and lumbar spinal canal see irregularly low-density shadow, extending to the direction of bilateral neural holes, CT value of about 6 6 Hu ~ 77Hu, uniform density, bilateral vertebral attachment destruction (see photo)