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患儿 女,18个月。主因反复发热,手足溃烂、坏死2月于1994年10月23日入院,家族史无可记叙,父母非近亲婚配。查体:双手、双足红肿,脱皮,以右侧为著,指趾端呈“鼓锤状”,不能伸屈。右足拇指外侧、底部及第二趾底部可见溃疡,有脓性渗出。右足拇指末节坏死,右第二、三趾趾甲脱落,颜色灰暗。双上肢自上臂中部以远,双下肢自大腿中部以远呈“手套、袜套”型痛觉减退。触觉存在。四肢肌力Ⅳ～Ⅴ级,肌张力正常。腱反射均未引出,病理征阴性。住院期间出现舌尖溃疡,经久不愈。实验室检查:血IgA792mg/L,IgG8190mg/L,IgM1530mg/L。右足正侧位片:右足各趾蹠骨骨质未见异常。神经传导速度:运动神经传导速度可见波幅降低,感觉神经传导速度:右下肢腓浅神经未引出,余均正常。听觉诱发电化:右耳听神经损伤,听力轻度下降。体感诱发电位:双下肢皮层电位未引出,余未见异常。
Children with children, 18 months. Mainly due to repeated fever, hand, foot ulceration, necrosis in February 23, 1994 admission, family history no recollection, parents non-relatives marriage. Physical examination: hands, feet swelling, peeling, with the right side, toe-end was “drum-shaped” can not flexion. Right foot outside the thumb, bottom and second toe visible ulcer bottom, purulent exudation. Right thumb distal necrosis, the second right, toe off, color darkness. Upper extremity from the middle of the upper arm far, lower extremity both legs from the middle of the thigh was “gloves, socks,” type pain relief. Tactile existence. Ⅳ ~ Ⅴ limb muscle strength, muscle tone normal. Tendon reflex did not lead to negative pathological signs. Tau mouth ulcers during hospitalization, prolonged healing. Laboratory tests: blood IgA792mg / L, IgG8190mg / L, IgM1530mg / L. Right foot is a positive lateral film: the right foot metatarsal bone no abnormalities. Nerve conduction velocity: motor nerve conduction velocity can be seen lower amplitude, sensory nerve conduction velocity: right lower extremity fibula shallow nerve did not lead, the remaining normal. Auditory evoked electrification: Right ear hearing nerve injury, mild hearing loss. Somatosensory evoked potential: double lower extremity cortex potential did not lead, I no abnormalities.