Erythromelalgia presenting with posterior reversible encephalopathy syndrome: A pediatric case report

Kengo Suzuki , Kazuhiro Uda , Mitsuru Tsuge , Kyosuke Arakawa , Kenji Shigehara , Takafumi Obara , Kosei Hasegawa , Hirokazu Tsukahara
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Abstract

Background

Erythromelalgia is a rare disorder characterized by erythema, warmth, and burning pain in the extremities. We report a pediatric case of erythromelalgia in a patient who developed posterior reversible encephalopathy syndrome (PRES), without any cutaneous signs.

Case presentation

A previously healthy 12-year-old girl presented to our pediatric clinic with burning extremity pain that had persisted for 6 weeks. The patient was treated with analgesics; however, the pain was refractory to these agents. Seven days after the first visit, she developed afebrile seizures and was transferred to our hospital. Her initial blood pressure was 139/105 mmHg (+2.0 SD), and brain magnetic resonance imaging revealed high intensity areas in the bilateral parietal and occipital lobes, leading to a diagnosis of PRES. Her blood pressure was difficult to control with anti-hypertensive agents. Burning pain in her extremities was relieved by cooling and worsened by warming. Although erythema was not observed in her hands or legs, erythromelalgia was suspected based on the characteristic nature of her pain. Intravenous lidocaine was administered for diagnosis, which was dramatically effective. After initiating mexiletine, the burning pain in her extremities disappeared, and hypertension improved. A final diagnosis of erythromelalgia with PRES was made.

Conclusion

A history of temperature-dependent pain relief and deterioration are important indicators of disease diagnosis, even if patients indicate a lack of erythema or warmth. Physicians should be aware that persistent pain due to erythromelalgia can lead to refractory hypertension and development of PRES.
红斑性肢痛表现为后部可逆性脑病综合征:一个儿科病例报告
背景:红斑性肢痛是一种罕见的疾病,其特征是四肢出现红斑、发热和灼痛。我们报告一个儿科病例的红斑性肢痛患者谁发展后可逆性脑病综合征(PRES),没有任何皮肤征象。病例介绍:一名既往健康的12岁女孩因持续6周的四肢灼烧性疼痛来到我们的儿科诊所。患者给予镇痛药治疗;然而,疼痛对这些药物是难治性的。第一次就诊后7天,患者出现发热性癫痫,转至我院。患者初始血压为139/105 mmHg (+2.0 SD),脑磁共振示双侧顶叶和枕叶高强度区,诊断为PRES,降压药难以控制血压。她四肢的灼痛在降温后减轻,在升温后加重。虽然在她的手或腿上没有观察到红斑,但根据她疼痛的特征,怀疑是红斑性肢痛症。静脉注射利多卡因进行诊断,效果显著。在开始使用美西汀后,她的四肢灼痛消失,高血压得到改善。最后诊断为红斑性肢痛伴PRES。结论温度依赖性疼痛缓解和恶化史是疾病诊断的重要指标,即使患者表现为缺乏红斑或温暖。医生应该意识到,红斑性肢痛症引起的持续疼痛可导致难治性高血压和PRES的发展。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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