网状青斑伴周围神经病变7例临床特征分析

Clinical features of peripheral neuropathy with livedo reticularis:an analysis of seven cases

回顾性分析2019年1月至2022年12月复旦大学附属华山医院神经科7例网状青斑伴周围神经病患者的临床特点、辅助检查、皮肤及神经病理特点并进行分析。7例患者中,5例为女性,2例为男性。周围神经病起病年龄(39.8±21.3)岁,病程(2.7±2.3)年。3例患者急性起病,4例患者慢性起病。所有患者均有肢体麻木,伴肢体无力6例,伴疼痛5例。神经电生理检查提示单神经病1例,多发单神经病2例,多发周围神经病2例,感觉神经元病2例。3例患者行皮肤活检,提示真皮层血管增生扩张,周围淋巴细胞浸润。3例患者行神经活检提示神经轴索损害。网状青斑伴周围神经病表现为肢体麻木无力,多数伴有疼痛。电生理改变形式多样。病理改变以轴索损害为主。

The clinical characteristics,auxiliary examinations,skin and neuropathological features of 7 patients who had reticular cyanosis with peripheral neuropathy from the Department of Neurology,Huashan Hospital,Fudan University from January 2019 to December 2022 were retrospectively analyzed.Among the 7 patients,5 were female and 2 were male.The age of onset of peripheral neuropathy was(39.8±21.3)years and the disease duration of peripheral neuropathy was(2.7±2.3)years.Three patients had acute onset and 4 patients had chronic onset.All the patients had limb numbness,with limb weakness in 6 patients and pain in 5 cases.Neuroelectrophysiological examination revealed 1 case of mononeuropathy,2 cases of polyneuropathy,2 cases of peripheral neuropathy,and 2 cases of sensory neuron neuropathy.Skin biopsy was performed in 3 patients,which presented hyperplasia and expansion of blood vessels in the dermis with lymphocyte infiltration.Nerve biopsy was performed in 3 patients,indicating axonal damage.Reticular cyanosis with peripheral neuropathy characterizes with numbness and weakness of limbs,most of which were accompanied by pain.Electrophysiological changes are in various forms.The pathological changes are dominated by the damage of axonal.