Ye Yan-Ting, Lu Jing-Fa, Wu Hui-Hui, Liu Juan-Hua, Zhao Yu-Kun, Luo Di-Qing
Department of Dermatology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, China.
Department of Dermatology, The First Affiliated Hospital of Gannan Medical College, Ganzhou, China.
Ther Adv Chronic Dis. 2022 May 19;13:20406223221099335. doi: 10.1177/20406223221099335. eCollection 2022.
Erythermalgia, a rare painful disorder, is characterized by recurrent pain attacking, warmth, and erythema that mainly involves the distal extremities. Red ear syndrome shares similar clinical features of erythermalgia afflicting the external ear with unilateral/bilateral distribution. The treatments of both diseases are still difficult without controlled therapeutics available up to date. A 12-year-old boy was referred because of 3 years of recurrent attacking of painful erythema and warmth that involved the ears alone, the episodes occurred several times daily with duration of dozens of minutes to hours for each flare. The symptoms could be relieved by cold water and triggered by heat stimuli as well as exciting and movement, and showed mild response to gabapentin, celecoxib, and topical lidocaine compounds in combination, but moderate to blocking injection of botulinum toxin to nervus auricularis magnus. However, systemic itraconazole 200 mg daily resulted in an excellent response after 5-week treatment, leading to milder erythema, warmth and burning sensation, shorter duration, and fewer relapses. The treatment continued for 6 months and then itraconazole was decreased to 100 mg daily for another 6 months until it was stopped, with maintenance of good conditions. In 3 months of follow-up after the treatment ceased, the patient had only 7 to 8 attacks over 10 days presenting as tolerable erythema that lasted for less than 10 min and relieved spontaneously, with absence of warmth and no need of treatment. We considered the patient to be a variant of erythermalgia rather than a red ear syndrome. The results showed that erythermalgia might involve the ears alone and itraconazole might be a potential agent for its treatment.
红斑性肢痛症是一种罕见的疼痛性疾病，其特征为反复出现的疼痛发作、发热和红斑，主要累及四肢远端。红耳综合征具有与红斑性肢痛症相似的临床特征，累及外耳，呈单侧/双侧分布。这两种疾病的治疗仍然困难，目前尚无有效的可控治疗方法。一名12岁男孩因3年来反复出现耳部疼痛性红斑和发热而前来就诊，发作仅累及耳部，每天发作数次，每次发作持续数十分钟至数小时。症状可通过冷水缓解，热刺激、兴奋和运动可诱发，联合使用加巴喷丁、塞来昔布和外用利多卡因复合制剂有轻度反应，但对耳大神经注射肉毒杆菌毒素有中度至阻滞作用。然而，每日口服200mg伊曲康唑，治疗5周后效果极佳，红斑、发热和烧灼感减轻，发作持续时间缩短，复发次数减少。治疗持续6个月，然后伊曲康唑减至每日100mg，再持续6个月直至停药，病情维持良好。治疗停止后3个月的随访中，患者在10天内仅发作7至8次，表现为可耐受的红斑，持续时间不到10分钟且可自行缓解，无发热，无需治疗。我们认为该患者是红斑性肢痛症的一种变异型而非红耳综合征。结果表明，红斑性肢痛症可能仅累及耳部，伊曲康唑可能是其潜在的治疗药物。
