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红斑性肢痛病成人少见,小儿更小见。该文描述1例小儿原发性红斑性肢痛病。病例17岁男孩,生后健康,无红斑性肢痛症家族史。7岁后出现双足疼痛和烧灼感,因逐渐加重并对镇痛药治疗无效而来诊。检查:患儿烦躁不安,双足背皮肤出现对称性发红,肿胀和紧张;双足背动脉搏动正常,无感觉障碍。上述表现当休息、冷敷和抬高患肢时减轻;而在加热和运动时加重。患区皮肤活检正常,血管呈正常组织学表现,无IgG、IgA、IgM、白蛋白、纤维蛋白原、C_1、C_3或C_4沉积。发病后第1个月出现短暂性血压升高(24/13.3kPa),经治疗后恢复正常,但其他表现没有改善。血铅浓度增高为0.57mg/L(正常值为0.3mg/L),尿铅浓度亦增高为0.7mg/L(正常值为0.5mg/L),但无铅中毒的其他特征性表现;经用螯合物疗法治疗后血铅和尿铅浓度恢复正常。在3年过程中对该例患儿曾采用非类固醇抗炎止痛剂、麻醉剂、利多卡因、坐骨神经阻滞、催眠药、抗抑郁药、抗组织胺药和镇静药等药物和方法进行治疗,但除有时出现短暂性足部灼痛消失外,其他表现改善不明显。
Rare limb pain in adults rare, children see less. This article describes a case of pediatric primary erythematous limb pain. A 17-year-old boy with a healthy family history of erythromelalgia. After the age of seven feet pain and burning sensation, due to gradual increase and the treatment of analgesics invalid visit. Check: irritability in children, bipedal symmetry appear redness, swelling and tension; bipedal normal beats, no sensory disturbances. The above symptoms relieve when resting, cold compressing and raising the affected limbs; while aggravating when heated and exercising. Skin biopsy of the affected area was normal, blood vessels were normal histological manifestations, no IgG, IgA, IgM, albumin, fibrinogen, C_1, C_3 or C_4 deposition. Transient elevated blood pressure (24 / 13.3 kPa) occurred in the first month after onset of symptoms and returned to normal after treatment, but the other symptoms did not improve. Blood lead levels increased 0.57mg / L (normal 0.3mg / L), urine lead concentrations also increased to 0.7mg / L (normal 0.5mg / L), but other characteristics of lead-free poisoning; Blood lead and urinary lead levels returned to normal after treatment with chelate therapy. The patient was treated with drugs and methods such as non-steroidal anti-inflammatory analgesics, anesthetics, lidocaine, sciatic nerve block, hypnotics, antidepressants, antihistamines and sedatives over a three year period. However, except for occasional transient burning of the foot disappeared, the other performance improvement was not obvious.