erythromelalgia

红斑痛
  • 文章类型: Case Reports
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  • 文章类型: Case Reports
    背景:红耳综合症是耳朵的灼烧感和红斑,与包括偏头痛在内的各种疾病相关,三叉神经痛,自身免疫性疾病等.RES病理生理学的理论已经从当前对合并症的理解中发展起来。表征RES的潜在机制对于定义有效治疗至关重要。
    方法:三名白种人患者,年龄15、47和67岁,偏头痛,本手稿报道了一例红斑性疼痛。RES病理生理学由于其可变的临床表现和许多合并症而尚未完全理解。很难确定有效的治疗方法。
    结论:RES似乎在很大程度上具有治疗抗性,大多数选择包括治疗相关疾病和减少疼痛。对未来案件的进一步调查应导致对RES的根本原因有更全面的了解,希望,成功的治疗。
    BACKGROUND: Red Ear Syndrome is a burning sensation and erythema of the ear, associated with a various number of disorders including migraine, trigeminal neuralgia, autoimmune disorders etc. Theories for RES pathophysiology have developed from current understandings of comorbid conditions. Characterizing the underlying mechanism of RES is crucial for defining effective treatments.
    METHODS: Three caucasian patients, ages 15, 47, and 67 years, with migraine, one with erythromelalgia are reported in this manuscript. RES pathophysiology is not fully understood due to its variable clinical presentation and numerous comorbid conditions, making it difficult to identify effective treatments.
    CONCLUSIONS: RES seems to be largely treatment-resistant, and most options involve treating the associated disorders and minimizing pain. Further investigation of future cases should lead to a more comprehensive understanding of the fundamental cause of RES and, hopefully, successful treatments.
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  • 文章类型: Case Reports
    本研究报告了一例奥希替尼诱导的转移性肺腺癌患者的红斑痛。奥希替尼是一种抗肿瘤药物,通过与细胞内受体酪氨酸激酶位点结合,不可逆地抑制表皮生长因子受体(EGFR)途径。从而阻止EGFR信号转导。一名77岁女性肺腺癌复发并继发转移,接受奥希替尼治疗。患者双手所有手指的远端指骨出现疼痛性红斑和温暖,随着热量的恶化和寒冷的缓解。根据临床数据,诊断为红血球痛。考虑到发病年龄,排除了原发性红血病.进一步的调查排除了红血病的其他次要原因,因此怀疑奥希替尼是病因.尽管尚未报道EGFR抑制剂诱导的红斑痛病例,EGFR抑制剂引起的皮肤不良事件已有文献报道.本病例可能是奥希替尼诱导的红斑痛的第一个证据,并可能帮助临床医生适当地支持发生这种EGFR抑制剂不良事件的患者。
    The present study reports a case of osimertinib-induced erythromelalgia in a patient with metastatic lung adenocarcinoma. Osimertinib is an antineoplastic drug that irreversibly inhibits the epidermal growth factor receptor (EGFR) pathway by binding to the intracellular receptor tyrosine kinase site, thus preventing EGFR signal transduction. A 77-year-old female with a lung adenocarcinoma recurrence with secondary metastases was prescribed osimertinib therapy. The patient presented with painful erythema and warmth in the distal phalanges of all fingers on both hands, which worsened with heat and relieved with cold. Based on clinical data, erythromelalgia was diagnosed. Considering the age of onset, a primary erythromelalgia was ruled out. Further investigations excluded other secondary causes of erythromelalgia, therefore osimertinib was suspected as the cause. Although no cases of EGFR inhibitor-induced erythromelalgia have been reported, cutaneous adverse events induced by EGFR inhibitors have been documented. The present case may be the first evidence of osimertinib-induced erythromelalgia and may help clinicians to properly support patients who develop this EGFR inhibitor adverse event.
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  • 文章类型: Journal Article
    雷诺综合征是许多自身免疫性疾病中的常见发现。准确诊断雷诺,并将其与模仿条件区分开来,在风湿病中势在必行。雷诺综合征和雷诺模仿者,尤其是痛苦的雷诺模仿者,可以证明执业风湿病学家的诊断挑战。痛苦的雷诺的模仿者会导致增加患者的压力和不必要的医疗工作;医疗保健提供者在评估患者对皮肤颜色变化和疼痛的担忧时需要了解雷诺的模仿者。本叙事综述旨在强调雷诺综合征,可以看到的重要痛苦的模仿者,诊断,和更新的管理建议。
    Raynaud\'s syndrome is a common finding in many autoimmune conditions. Accurately diagnosing Raynaud\'s, and differentiating it from mimicking conditions, is imperative in rheumatologic diseases. Raynaud\'s syndrome and Raynaud\'s mimickers, especially painful Raynaud\'s mimickers, can prove a diagnostic challenge for the practicing rheumatologist. Painful Raynaud\'s mimickers can lead to increased patient stress and unnecessary medical work up; Healthcare providers need to be aware of Raynaud\'s mimickers when evaluating patient concerns of skin color changes and pain. The present narrative review aims to highlight Raynaud\'s syndrome, important painful mimickers that may be seen, diagnosis, and updated management recommendations.
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  • 文章类型: Case Reports
    红血病是一种罕见的,以严重的不适发作为特征的高度衰弱性疾病,红斑,四肢脱皮。其原因包括遗传因素,药物,和几个潜在的医疗条件。本文通过一例病例报告和文献复习,描述了一种新的红血球痛病因。
    一名47岁的白人男子,有两年的间歇性疼痛史,发红和脱皮的手。他经历了几次这样的事件,每次持续3-4周。皮肤活检证实了红血球痛的诊断。在几次复发之后,他承认间歇性使用伪麻黄碱作为鼻减充血剂,这与红斑痛的发作相吻合。在停止该药物治疗后报告症状完全缓解。
    据报道,伪麻黄碱会引起广泛的皮肤反应,但尚未发现伪麻黄碱会导致红斑。认识到这种罕见的副作用可以提供早期诊断和降低发病率。
    UNASSIGNED: Erythromelalgia is a rare, highly debilitating disorder characterised by severe episodes of discomfort, erythema, and desquamation of the extremities. Its causes include genetic factors, medications, and several underlying medical conditions. This paper describes a novel cause of erythromelalgia through a case report and literature review.
    UNASSIGNED: A 47-year-old Caucasian man presented with a two-year history of intermittent pain, redness and desquamation of the hands. He experienced several such episodes, each lasting 3-4 weeks. A skin biopsy confirmed the diagnosis of erythromelalgia. After several recurrences, he admitted to the intermittent use of pseudoephedrine as a nasal decongestant, which coincided with the episodes of erythromelalgia. Complete resolution of symptoms was reported on cessation of this medication.
    UNASSIGNED: Pseudoephedrine has been reported to cause a wide range of cutaneous reactions but has not been known to precipitate erythromelalgia. Recognition of this rare side effect may offer early diagnosis and reduced morbidity.
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  • 文章类型: Journal Article
    红血病,有主要和次要演示,导致热量,疼痛,皮肤发红.这种情况似乎有自主神经的基础,血管舒缩功能障碍导致某些血管扩张和其他血管收缩。尚未报道持续有效的治疗方法。抗惊厥药,抗抑郁药,抗组胺药,抗炎,抗高血压药,镇痛药,营养,已经尝试了局部方法,就像利多卡因输注一样,神经阻滞,胸腰椎交感神经切除术.骨间膜刺激似乎会影响所治疗肢体的局部自主神经环境。这种方法用于患有红血病的患者。
    一名36岁的红斑患者接受骨间膜刺激治疗。在1年的时间范围内以1-3个月的间隔给予八种治疗。
    这个病人反复经历了从她的灼热感觉异常缓解,肿胀,排汗,每次治疗后她的四肢红润变色6-8小时。随着时间的推移,她的不适感逐渐消退。
    骨间膜刺激是安全的,简单,和红斑痛的有效治疗方法,这是众所周知的难以治疗。该患者对治疗的反应可能是其自主神经系统局部紊乱的结果。肢体自主神经环境的操纵可能是骨间膜刺激作用机制的重要因素。
    UNASSIGNED: Erythromelalgia, which has primary and secondary presentations, causes heat, pain, and redness in the skin. The condition seems to have an autonomic basis, with vasomotor dysfunction causing dilatation of some blood vessels and constriction of others. No consistently effective treatments have been reported. Anticonvulsant, antidepressant, antihistamine, anti-inflammatory, antihypertensive, analgesic, nutritional, and topical approaches have been tried as were lidocaine infusions, nerve blocks, and thoracic and lumbar sympathectomies. Interosseous membrane stimulation appears to affect the local autonomic milieu in the extremity being treated. This approach was used on a patient with erythromelalgia.
    UNASSIGNED: A 36-year-old woman with erythromelalgia was treated with interosseous membrane stimulation. Eight treatments were given over a 1-year timeframe at 1-3-month intervals.
    UNASSIGNED: This patient repeatedly experienced much relief from her burning paresthesias, swelling, diaphoresis, and ruddy discoloration of her extremities for 6-8 hours following each treatment. The intensity of her discomfort subsided gradually over time.
    UNASSIGNED: Interosseous membrane stimulation is a safe, simple, and effective treatment for erythromelalgia, which is notoriously refractory to treatment. This patient\'s response to treatment might have been a result of localized derangement of her autonomic nervous system. It is possible that manipulation of the autonomic milieu of an extremity is a significant factor in the mechanism of action of interosseous membrane stimulation.
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  • 文章类型: Case Reports
    红花痛是一种病因不明的罕见综合征。无论是主要的还是次要的,这种情况的特点是阵发性红斑发作,疼痛,和四肢的热量。我们报告了在开始英夫利昔单抗治疗后发生的两例红斑痛。第一例病例涉及一名38岁的患者,自2022年8月以来一直随访回肠结肠克罗恩病,根据蒙特利尔分类法分类为A2L3B3。对治疗耐药,需要英夫利昔单抗治疗。首次输注英夫利昔单抗两个月后,患者出现红斑痛症状。在通过病因评估和进行药理学研究排除其他潜在原因后,英夫利昔单抗被认为是最可能的原因.英夫利昔单抗停用,并开始对症治疗,包括血管激光会议。患者表现出显著的临床改善。在第二种情况下,1例根据蒙特利尔分类分类为A1L3B3的16岁回肠结肠克罗恩病患者接受回盲部切除术治疗,并接受英夫利昔单抗输注.第二次输液16天后,她出现了红血病的临床症状。病因评估尚无定论。由于强烈怀疑继发于肿瘤坏死因子(TNF)α抑制剂治疗的红斑痛,英夫利昔单抗被替换为ustekinumab.患者还接受了对症治疗,她的临床状况有所改善,以疼痛消失为标志。
    Erythromelalgia is a rare syndrome with a generally unknown etiology. Whether primary or secondary, this condition is characterized by paroxysmal episodes of erythema, pain, and heat in the extremities. We report two cases of erythromelalgia occurring after the initiation of treatment with infliximab. The first case involves a 38-year-old patient who had been followed since August 2022 for ileocolonic Crohn\'s disease classified as A2L3B3 according to the Montreal classification, which was resistant to treatment and required infliximab therapy. Two months after the first infusion of infliximab, the patient developed symptoms of erythromelalgia. After ruling out other potential causes through an etiological assessment and conducting a pharmacological investigation, infliximab was considered the most likely cause. Infliximab was discontinued, and symptomatic treatment was initiated, including vascular laser sessions. The patient showed significant clinical improvement. In the second case, a 16-year-old patient with ileocolonic Crohn\'s disease classified as A1L3B3 according to the Montreal classification was treated with ileocecal resection and received an infusion of infliximab. Sixteen days after the second infusion, she developed clinical symptoms of erythromelalgia. The etiological assessment was inconclusive. Due to a strong suspicion of erythromelalgia secondary to tumor necrosis factor (TNF) alpha inhibitor therapy, infliximab was replaced with ustekinumab. The patient also received symptomatic treatment, and her clinical condition improved, marked by the disappearance of pain.
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  • 文章类型: Journal Article
    红斑痛(EM)是一种罕见的疾病,它的特征仍然很差。在本论文中,我们比较了一名女性EM患者的手部灌注,在挑战之下,健康的对照组。使用带有集成热探头的激光多普勒流量计(LDF),测量是在休息时(I期)和两个单独的挑战后,一只手臂闭塞性充血(PORH)(A)和一只手用冰降低皮肤温度(冷却)(B)(II期)。最终测量周期对应于恢复(阶段III和IV)。对照组包括10名健康女性(27.3±7.9岁)。在开始使用β-受体阻滞剂(6.25mg卡维地洛,每天两次)的新治疗方法后一个月,在EM患者中进行第二组测量。使用小波变换(WT)分析评估患者LDF的Z评分和与对照组相比的温度波动。这里,|Z|>1.96的波动被认为与健康值显著不同,而正或负Z值表示与对照平均值的较高或较低偏差。冷却引起更多可测量的LDF和温度波动变化,特别是在较高的频率分量(心脏,呼吸,和肌源性),而PORH尤其引起低频成分的变化(肌源性,自主性,和内皮)。在第二次测量中没有观察到明显的Z分数偏差,这可能意味着在新的治疗方法后患者的远端灌注稳定。这项分析涉及一名EM患者,虽然显然是探索性的,与健康组相比,WT衍生的生理成分值显示出显着偏差,确认有兴趣使用低温作为挑战者。与临床评估达成的明显共识为将这种方法扩展到血管医学中的其他患者和病理提供了可能性。
    Erythromelalgia (EM) is a rare disease, which is still poorly characterized. In the present paper, we compared the hand perfusion of one female EM patient, under challenges, with a healthy control group. Using a laser Doppler flowmeter (LDF) with an integrated thermal probe, measurements were taken in both hands at rest (Phase I) and after two separate challenges-post-occlusive hyperemia (PORH) in one arm (A) and reduction of skin temperature (cooling) with ice in one hand (B) (Phase II). The final measurement periods corresponded to recovery (Phases III and IV). The control group involved ten healthy women (27.3 ± 7.9 years old). A second set of measurements was taken in the EM patient one month after beginning a new therapeutic approach with beta-blockers (6.25 mg carvedilol twice daily). Z-scores of the patient\'s LDF and temperature fluctuations compared to the control group were assessed using the Wavelet transform (WT) analysis. Here, fluctuations with |Z| > 1.96 were considered significantly different from healthy values, whereas positive or negative Z values indicated higher or lower deviations from the control mean values. Cooling elicited more measurable changes in LDF and temperature fluctuations, especially in higher frequency components (cardiac, respiratory, and myogenic), whereas PORH notably evoked changes in lower frequency components (myogenic, autonomic, and endothelial). No significant Z-score deviations were observed in the second measurement, which might signify a stabilization of the patient\'s distal perfusion following the new therapeutic approach. This analysis involving one EM patient, while clearly exploratory, has shown significant deviations in WT-derived physiological components\' values in comparison with the healthy group, confirming the interest in using cold temperature as a challenger. The apparent agreement achieved with the clinical evaluation opens the possibility of expanding this approach to other patients and pathologies in vascular medicine.
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