Melkersson-Rosenthal syndrome

Melkersson - Rosenthal 综合征
  • 文章类型: Case Reports
    背景:Melkersson-Rosenthal综合征(MRS)是一种罕见的神经粘膜皮肤疾病,以复发性水肿为特征,面部麻痹,和通常与plicata舌相关的神经功能障碍。虽然MRS的病因尚不清楚,越来越多的证据表明自身免疫参与。
    方法:本文报道一例25岁男性,以MRS为首发症状,其次是颞下颌关节骨关节炎(TMJ-OA)。包括手术在内的综合诊断和多学科治疗方法,局部注射,并实施了口服药物治疗,导致良好的预后。
    结论:这些发现支持MRS是由自身免疫引起的全身性肉芽肿病的假设,也可能通过免疫相关机制影响TMJ-OA的发生和发展。这项研究强调了全身免疫调节在MRS和TMJ-OA合并症患者治疗中的意义。
    BACKGROUND: Melkersson-Rosenthal syndrome (MRS) is a rare neuro-mucocutaneous disorder characterized by recurrent edema, facial palsies, and nerve dysfunctions often associated with the plicata tongue. Although the etiology of MRS is not well understood, there is growing evidence suggesting an autoimmune involvement.
    METHODS: This paper presents a case report of a 25-year-old male with MRS as the initial symptom, followed by temporomandibular joint osteoarthritis (TMJ-OA). A comprehensive diagnosis and multidisciplinary treatment approach including surgery, local injections, and oral medication were implemented, resulting in a favorable prognosis.
    CONCLUSIONS: These findings support the hypothesis that MRS is a systemic granulomatous disease caused by autoimmunity, which may also influence the occurrence and development of TMJ-OA through immune-related mechanisms. This study emphasizes the significance of systemic immune regulation in the treatment of patients with MRS and TMJ-OA comorbid conditions.
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  • 文章类型: Case Reports
    背景:肉芽肿性唇炎是一种罕见的疾病,特发性,和无痛的嘴唇肿胀。诊断通过组织病理学检查证实。病因未知和对潜在机制知之甚少导致难以建立有效的治疗方法。此案例研究提出了射频疗法在难治性肉芽肿性唇炎治疗中的有效性。
    方法:一名68岁的患者表现为下唇肥大和肿胀,活检显示光化性唇炎.患者接受了嘴唇剃刮和前移粘膜瓣,明确的组织学检查证实了肉芽肿性唇炎的诊断。没有观察到口面部肉芽肿的其他征象,补充病因研究对全身性疾病阴性。嘴唇肿胀再次出现并持续存在,影响了生活质量.因此,在嘴唇的粘膜下层和真皮下层进行射频治疗,导致显着的美学和功能改善,五年后没有进一步复发。
    结论:肉芽肿性唇炎的治疗具有挑战性。在严重病例中,目前的主要治疗方法是皮质治疗或减少唇缘成形术。射频有潜力作为一种治疗方案,呈现值得持久的功能和美学结果,发病率最低。
    BACKGROUND: Granulomatous cheilitis is a rare disorder characterized by recurrent, idiopathic, and painless lip swelling. The diagnosis is proven by histopathological examination. The unknown aetiology and poorly understood underlying mechanism contribute to the difficulty in establishing an effective treatment. This case study proposes the effectiveness of radiofrequency therapy in the management of refractory granulomatous cheilitis.
    METHODS: A 68-year-old patient presented with hypertrophy and swelling of the lower lip, and a biopsy revealed actinic cheilitis. The patient underwent lip shaving and an advancement mucosal flap, and definitive histologic examination confirmed the diagnosis of granulomatous cheilitis. No other signs of orofacial granulomatosis were observed, and the complementary aetiological study was negative for systemic disease. The lip swelling reappeared and persisted, which interfered with the quality of life. Hence, radiofrequency therapy was performed in the submucosal and subdermal layers of the lip, resulting in significant aesthetic and functional improvement and no further relapses after five years.
    CONCLUSIONS: The management of granulomatous cheilitis is challenging. The current mainstay treatment is corticotherapy or reduction cheiloplasty in severe cases. Radiofrequency has potential as a treatment option in debilitating macrocheilia, presenting worthy long-lasting functional and aesthetical results, with minimal morbidity.
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  • 文章类型: Case Reports
    Melkersson-Rosenthal综合征是一种病因不明的罕见疾病。它的特点是一个经典的三合会的症状:复发面部和嘴唇肿胀,面部麻痹和舌头裂开。我们报告了一例29岁的女性患者,其表现出上述Melkersson-Rosenthal综合征的症状。然而,临床检查显示异常表现,这是牙龈增生。通过全身性类固醇和牙龈增生的手术切除部分控制了症状。从我们的病例中得出的最重要的发现是,牙龈肿大可以被确定为MRS疾病的罕见临床特征,这被证实是难以管理的。
    Melkersson-Rosenthal syndrome is a rare condition of unknown etiology. It is characterized by a classical triad of symptoms: relapsing facial and lip swelling, facial palsy and a fissured tongue. We report the case of a 29-year-old female patient who presented with the above-mentioned symptoms of Melkersson-Rosenthal syndrome. However, clinical examination revealed an exceptional manifestation, which is the gingival hyperplasia. The symptoms were partially managed with systemic steroids and surgical resection of gingival hyperplasia. The most significant finding to emerge from our case is that gingival enlargement can be identified as a rare clinical feature of the MRS disease, which is confirmed difficult to be managed.
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  • 文章类型: Case Reports
    Melkersson-Rosenthal syndrome (MRS) is a rare neuro-mucocutaneous condition that presents with orofacial swelling, facial paralysis, and a fissured tongue. These classic triad of symptoms, however, very rarely present simultaneously. The symptoms are often seen alone or in pairs and appear at any stage in life. Although the etiology of this condition is unknown, various contributing factors have been suggested including infections, immune deficiencies, stress, and genetic predispositions. We present a case of a 23-year-old female patient who has a longstanding history of MRS, anxiety, and depression, and who attempted to overdose on prescription medications due to suicidal ideations.
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  • 文章类型: Case Reports
    口面部肉芽肿是一种临床表现为无痛性阴唇增大的疾病,口周和粘膜水肿,口腔溃疡,和牙龈炎.它的特征是口腔和颌面部区域的非坏死性肉芽肿性炎症。当肿胀只影响嘴唇时,这种病理被称为Miescher肉芽肿性唇炎;然而,当它还会导致面部麻痹和舌头重叠时,它被称为Melkersson-Rosenthal综合征。我们报告了一例成功应用局部(病灶内)类固醇联合治疗的病例,全身性抗生素,和全身性类固醇。经过6个月的治疗,我们观察到牙龈增生,颊粘膜和唇水肿的改善。
    Orofacial granulomatosis is a condition that manifests clinically as painless labial enlargement, perioral and mucosal edema, oral ulcers, and gingivitis. It is characterized by non-necrotizing granulomatous inflammation of the oral and maxillofacial region. When the swelling only affects the lips, the pathology is called Miescher\'s granulomatous cheilitis; however, when it also causes facial paresis and lingua plicata, it is known as Melkersson-Rosenthal syndrome. We report a case that was successfully treated with a combination of a local (intralesional) steroid, a systemic antibiotic, and a systemic steroid. After 6 months of therapy, we observed improvement in gingival hyperplasia and buccal mucosa and lip edema.
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  • 文章类型: Case Reports
    Melkersson-Rosenthal综合征(MRS)是一种发病机制未知的神经粘膜皮肤疾病。通过这种交流,我们描述了1例MRS完全的26岁女性患者,该患者通过下一代测序在肿胀的唇活检中检测到了偶然分枝杆菌.病灶内注射糖皮质激素联合广谱抗生素后,患者症状稍有改善,而在进一步治疗龋齿和去除残根后,它们得到了显着改善。该病例提供了对MRS可能的微生物感染发病机制的见解,推测M.fortuitum与肉芽肿和神经元疾病有关,很可能来自牙源性。
    Melkersson-Rosenthal syndrome (MRS) is a neuromucocutaneous disease of unknown pathogenesis. With this communication, we describe a case of a 26-year-old woman with complete MRS in whom Mycolicibacterium fortuitum was detected in the swelling lip biopsy by next- generation sequencing. The patient\'s symptoms were slightly improved after intralesional corticosteroid injection combined with broad-spectrum antibiotics, while they were significantly improved after further treatment of dental caries and removal of the residual root. This case provides insight into the possible microbial infection pathogenesis of MRS, and M. fortuitum was speculated to be related to granulomatous and neuronal disorders, most probably from odontogenic origin.
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  • 文章类型: Case Reports
    Melkersson-Rosenthal综合征(MRS)是一种罕见的神经粘膜皮肤疾病,临床特征为复发性面神经麻痹的三联征,口面部肿胀,裂开的舌头。本报告介绍了根据其三个临床特征诊断为MRS的38岁女性病例。使用皮质类固醇(口服泼尼松龙1mg/kg/天)治疗一周,然后在两周内逐渐减少剂量。要求定期进行年度长期随访以监测疾病活动。
    Melkersson-Rosenthal syndrome (MRS) is an uncommon neuro-mucocutaneous disease, clinically characterized by a triad of recurrent facial palsy, orofacial swelling, and fissured tongue. This report presents the case of a 38-year-old female diagnosed with MRS based on its three clinical features. A corticosteroid (1 mg/kg/day of oral prednisolone) was prescribed for a week, and then tapered off over two weeks by gradually lowering the dose. Regular annual long-term follow-ups were requested to monitor the disease activity.
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  • 文章类型: Case Reports
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    文章类型: Case Reports
    肉芽肿性唇炎或Miescher唇炎是一种罕见的肉芽肿性疾病,其定义为反复出现的嘴唇肿胀或其他面部软组织水肿。组织病理学显示非干酪样肉芽肿和多核巨细胞。确切的病因未知,虽然遗传背景,免疫不规则,全身性或传染性疾病有助于疾病的发作。没有治疗指南。通常的治疗选择包括全身性或病灶内皮质类固醇,一系列抗生素,和免疫抑制剂.一名63岁的患者出现嘴唇肿胀和其他面部软组织同时肿胀,被诊断为肉芽肿性唇炎。症状发生在SARS-CoV-2感染后3周。全身性皮质类固醇和抗组胺药的初始治疗不足。在这里,我们报告了多西环素和甲硝唑联合治疗的成功方法。
    Granulomatous cheilitis or Miescher\'s cheilitis is a rare granulomatous disorder defined by recurrent lip swelling or edema of other facial soft tissues. Histopathology shows non-caseous granulomas and multinucleated giant cells. The exact etiology is unknown, although genetic background, immunological irregularities, and systemic or infectious diseases contribute to the onset of disease. There are no treatment guidelines. The usual treatment options include systemic or intralesional corticosteroids, a spectrum of antibiotics, and immunosuppressants. A 63-year-old patient presenting with lip swelling and simultaneous swelling of other facial soft tissues was diagnosed with granulomatous cheilitis. The symptoms occurred 3 weeks after SARS-CoV-2 infection. Initial treatment with systemic corticosteroids and antihistamines was inadequate. Here we report successful treatment with a combination of doxycycline and metronidazole.
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