Department of Ophthalmology, IRCCS Humanitas Research Hospital, Milan, Italy.
Department of Biomedical Sciences, Humanitas University, Milan, Italy.
Am J Case Rep. 2024 Jun 14;25:e943421. doi: 10.12659/AJCR.943421.
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.
Morbihan 病,又称 Morbihan 综合征,是一种罕见的医学病症,其特征为慢性面部水肿,主要影响面部的上三分之二。尽管这种病症在医学文献中已被认识了几十年,但它的真实流行程度和潜在病理生理学仍未被充分了解。各种假说,包括淋巴引流受损、血管通透性异常、免疫失调以及对螨虫感染的炎症反应,都被提出以解释其病因。
我们报告了一例 61 岁男性患者,其面部上三分之一出现有组织的眼周水肿,最终诊断为 Morbihan 病。患者接受了中面部提升术,以便从眼睑水肿皮肤中获取组织进行组织病理学检查,结果显示慢性炎症、小淋巴管扩张以及螨虫在滤泡内定位的特征。这些发现不具有特异性,但与诊断假说一致。患者被转介给风湿病医生进行进一步评估和治疗。他对全身皮质类固醇和免疫抑制治疗反应不佳,反而导致水肿扩展到上眼睑。患者选择不进行进一步治疗。
Morbihan 病由于其罕见性和与其他面部病症的重叠临床特征,常常被误诊。其治疗具有挑战性,可能需要结合药物和手术干预。全身皮质类固醇、免疫抑制剂和局部治疗都取得了不同程度的成功。在严重病例中,可以考虑手术程序,如眼睑成形术或激光治疗。早期识别和适当的治疗对于改善患者的预后和生活质量至关重要。