莫尔比昂病:一例罕见的眶周双侧水肿病例,组织病理学发现为慢性炎症和脱霉菌定位。

IF 1 Q3 MEDICINE, GENERAL & INTERNAL American Journal of Case Reports Pub Date : 2024-06-14 DOI:10.12659/AJCR.943421
Alessandra Di Maria, Filippo Confalonieri, Gianmaria Barone, Sofia Manara, Vanessa Ferraro
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引用次数: 0

摘要

背景 莫尔比昂病又称莫尔比昂综合征,是一种罕见的内科疾病,主要表现为面部上三分之二处的慢性水肿。尽管数十年来医学文献一直承认这种疾病,但对其真正的发病率和潜在的病理生理学仍知之甚少。人们提出了各种假说来解释其病因,包括淋巴引流受损、血管通透性异常、免疫调节失调和脱虱侵袭引起的炎症反应。病例报告 我们报告了一例 61 岁男性的病例,患者面部上三分之一处出现有组织的眼周水肿,最终被诊断为莫尔比昂病。患者接受了中面部提升术,可以取回组织对眼睑水肿皮肤进行组织病理学检查,结果发现了慢性炎症、小淋巴管异位和脱毛癣菌在眼泡内定位的特征。这些结果并不特异,但与诊断假设相符。患者被转诊到风湿免疫科接受进一步评估和治疗。他对全身皮质类固醇激素和免疫抑制疗法反应不佳。这反而导致水肿扩展到上眼睑。患者选择不再接受进一步治疗。结论 莫尔比昂病因其罕见性和与其他面部疾病重叠的临床特征而经常被误诊。该病的治疗极具挑战性,可能需要结合药物和手术治疗。全身使用皮质类固醇激素、免疫抑制剂和局部治疗的效果各不相同。严重病例可考虑进行眼睑成形术或激光治疗等外科手术。早期识别和适当治疗对改善患者的预后和生活质量至关重要。
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Morbihan Disease: A Rare Case of Periorbital Bilateral Edema with Histopathological Findings of Chronic Inflammation and Demodex Localization.

BACKGROUND Morbihan disease, also known as Morbihan syndrome, is a rare medical condition characterized by chronic facial edema predominantly affecting the upper two-thirds of the face. Despite being recognized in medical literature for decades, its true prevalence and underlying pathophysiology remain poorly understood. Various hypotheses, including impaired lymphatic drainage, abnormal vascular permeability, immune dysregulation, and inflammatory reactions to demodex infestation, have been proposed to explain the etiology. CASE REPORT We present a case of a 61-year-old man with organized periocular edema of the upper third of the face, ultimately leading to Morbihan disease diagnosis. The patient underwent a midface lift, allowing for tissue retrieval for histopathological examination of the eyelid edematous skin, which revealed chronic inflammation, ectasia of small lymphatic vessels, and features of demodex intrafollicular localization. These findings were not specific, but consistent with the diagnostic hypothesis. The patient was referred to a rheumatologist for further evaluation and treatment. He did not respond well to systemic corticosteroids and immunosuppressive therapy. Rather, this resulted in extension of the edema to the upper eyelid. The patient opted not to undergo further treatment. CONCLUSIONS Morbihan disease is often misdiagnosed due to its rarity and overlapping clinical features with other facial conditions. Its management is challenging and can require a combination of medical and surgical interventions. Systemic corticosteroids, immunosuppressive agents, and topical treatments have had varying success. Surgical procedures, such as blepharoplasty or laser therapy, can be considered in severe cases. Early recognition and appropriate management are crucial to improving patient outcomes and quality of life.

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来源期刊
American Journal of Case Reports
American Journal of Case Reports Medicine-Medicine (all)
CiteScore
1.80
自引率
0.00%
发文量
599
期刊介绍: American Journal of Case Reports is an international, peer-reviewed scientific journal that publishes single and series case reports in all medical fields. American Journal of Case Reports is issued on a continuous basis as a primary electronic journal. Print copies of a single article or a set of articles can be ordered on demand.
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