• 文章类型: Case Reports
    布劳综合征(BS),是一种自身炎症性肉芽肿病,其特征是皮肤有明显的三联征,接头,和结节病类似的眼部疾病,但在结节病中经常观察到的肺部受累很少。BS患者的肉芽肿表现出明显的形态,表明慢性炎症反应旺盛。BS患者可能有肉芽肿性肺病变,这需要早期诊断。为了确定是否需要对肺部病变进行治疗干预,检查经支气管镜肺冷冻活检标本并积累肺部受累的BS病例可能有助于将来改善BS管理。
    Blau syndrome (BS), is an autoinflammatory granulomatosis disease characterized by a distinct triad of skin, joint, and eye disorders similar to those of sarcoidosis, but the lung involvement frequently observed in sarcoidosis are rare. Granulomas from patients with BS displayed a distinct morphology indicating an exuberant chronic inflammatory response. Patients with BS may have granulomatous lung lesions, which require early diagnosis. To determine whether therapeutic intervention is needed for lung lesions, examining transbronchial lung cryobiopsy specimens and accumulating cases of BS with lung involvement could be contributed to improving BS management in the future.
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  • 文章类型: Case Reports
    丝虫病是印度等热带和亚热带国家的主要公共卫生问题,Wuchereriabancrofti占淋巴丝虫病的90%。很少观察到由免疫系统与微丝菌及其扩散产物相互作用引起的额外淋巴表现。在涉及的各种器官中,脾受累是丝虫病的一种罕见的额外淋巴表现,当与已知的恶性肿瘤或淋巴瘤等原发性恶性肿瘤相关时,可在临床上伪装为转移。特此,我们报道了一例罕见的脾丝虫病合并胰腺实性假乳头状上皮性肿瘤的病例,该病例发生在一名20岁女性患者中,并伴有外周血嗜酸性粒细胞增多.
    Filariasis is a major public health concern in tropical and subtropical countries like India with Wuchereria bancrofti accounting for 90% of lymphatic filariasis. Rarely observed are extra lymphatic manifestations caused by interaction of immune system with microfilaria and their diffusible products. Among various organs involved, splenic involvement is a rare extra lymphatic manifestation of filariasis and can masquerade clinicoradiologically as metastasis when associated with a known malignancy or as a primary malignancy like lymphoma. Hereby, we present an unusual case of coincidence of splenic filariasis with pancreatic solid pseudopapillary epithelial neoplasm in a 20-year-old woman associated with peripheral blood eosinophilia.
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  • 文章类型: Case Reports
    背景:肉芽肿性多血管炎(GPA)是抗中性粒细胞胞浆抗体(ANCA)相关性血管炎的最常见形式之一。GPA的组织学特征为除血管炎外的坏死性肉芽肿性炎症。GPA的诊断取决于临床表现,ANCA阳性的血清学证据,和/或坏死性血管炎或肉芽肿性破坏性实质性炎症的组织学证据。细胞质ANCA(c-ANCA)在65%-75%的GPA患者中呈阳性,伴有蛋白酶3(PR3),c-ANCA的主要靶抗原,另有5%的GPA患者ANCA阴性.
    方法:患者,一个52岁的男性,出现无法解释的鼻塞,耳鸣,和听力损失。在经历了4个月的这些症状后,病人随后出现发烧和头痛。影像学检查显示存在双侧耳乳突炎和部分副鼻窦炎,ANCA结果为阴性。抗感染治疗被证明是无效的,但患者的症状和发烧迅速缓解后1周的甲基强的松龙40毫克,每天一次。然而,连续使用甲基强的松龙片剂3个月后,患者出现了伴有右侧偏头痛的发热复发,c-ANCA和PR3阳性,脑脊液中总蛋白增加。患者被诊断为GPA。在接受静脉注射甲基强的松龙40mg/d和环磷酰胺0.8g每月的治疗方案后,患者出现发热和头痛缓解。此外,ANCA水平为阴性,且无复发.
    结论:对于ANCA阴性的GPA患者,有可能早期漏诊.组织病理学结果和多学科交流的整合在促进ANCA阴性GPA中起着至关重要的作用。
    BACKGROUND: Granulomatosis with polyangiitis (GPA) is one of the most prevalent forms of the antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. GPA is characterized histologically by necrotizing granulomatous inflammation in addition to vasculitis. The diagnosis of GPA depends on clinical presentation, serological evidence of a positive ANCA, and/or histological evidence of necrotizing vasculitis or granulomatous destructive parenchymal inflammation. Cytoplasmic ANCA (c-ANCA) is positive in 65%-75% of GPA patients, accompanied by proteinase 3 (PR3), the main target antigen of c-ANCA, another 5% of GPA patients had negative ANCA.
    METHODS: The patient, a 52-year-old male, presented with unexplained nasal congestion, tinnitus, and hearing loss. After a duration of 4 months experiencing these symptoms, the patient subsequently developed fever and headache. The imaging examination revealed the presence of bilateral auricular mastoiditis and partial paranasal sinusitis, and the ANCA results were negative. The anti-infective therapy proved to be ineffective, but the patient\'s symptoms and fever were quickly relieved after 1 wk of treatment with methylprednisolone 40 mg once a day. However, after continuous use of methylprednisolone tablets for 3 months, the patient experienced a recurrence of fever accompanied by right-sided migraine, positive c-ANCA and PR3, and increased total protein in cerebrospinal fluid. The patient was diagnosed with GPA. After receiving a treatment regimen of intravenous methylprednisolone 40 mg/d and cyclophosphamide 0.8 g monthly, the patient experienced alleviation of fever and headache. Additionally, the ANCA levels became negative and there has been no recurrence.
    CONCLUSIONS: For GPA patients with negative ANCA, there is a potential for early missed diagnosis. The integration of histopathological results and multidisciplinary communication plays a crucial role in facilitating ANCA-negative GPA.
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  • 文章类型: Case Reports
    血管内治疗后的异物肉芽肿是罕见的并发症,主要在脑或皮肤血管组织中报道。据我们所知,迄今为止,尚无研究报道将N-丁基-2-氰基丙烯酸酯(NBCA)-碘油混合物注入腹部动脉后发生腹部异物肉芽肿.这项研究报告了一例异物肉芽肿,在使用NBCA-碘油混合物栓塞右髂内动脉瘤12个月后出现,这对区分恶性肿瘤提出了挑战。我们介绍了一名77岁的男子,他接受了右髂内动脉瘤的栓塞和腹主动脉瘤的开放手术修复。术后12个月进行的对比增强CT检查显示右侧腹膜后肿块围绕髂腰肌。质量包含多个,小,高密度区域,提示NBCA-碘油混合物从栓塞的右髂内动脉瘤转移。鉴别诊断包括异物肉芽肿,淋巴瘤和肉瘤.病变的活检显示肉芽肿具有不同的炎症阶段,没有铁血黄素沉积,多核巨细胞,和含有脂肪的泡沫细胞,被诊断出异物肉芽肿.对微生物的特殊染色未发现提示感染的发现。因为病人没有症状,未进行治疗。术后24个月的CT造影显示肿块缩小,术后48个月未发现大小变化。本报告重点介绍了一种模仿恶性肿瘤的异物肉芽肿。NBCA-碘油混合物铸型的血管外迁移可能导致肉芽肿形成。放射科医师应考虑使用NBCA栓塞到腹部动脉后的异物肉芽肿。
    Foreign body granulomas following endovascular treatment are rare complications and are mostly reported in the brain or cutaneous vascular tissues. To the best of our knowledge, no study to date has reported on foreign body granulomas in the abdomen after injection of N-butyl-2-cyanoacrylate (NBCA)-lipiodol mixture into the abdominal arteries. This study reports a case of foreign body granuloma that appeared 12 months after the embolization of a right internal iliac artery aneurysm using an NBCA-lipiodol mixture, which posed challenges in differentiation from malignant tumors. We present a 77-year-old man who underwent embolization of a right internal iliac artery aneurysm and open surgical repair of an abdominal aortic aneurysm. A contrast-enhanced CT performed 12 months postoperatively revealed a right-sided retroperitoneal mass surrounding the iliopsoas muscle. The mass contained multiple, small, hyperdense areas, suggesting the migration of the NBCA-lipiodol mixture casts from the embolized right internal iliac artery aneurysm. The differential diagnosis included foreign body granuloma, lymphoma, and sarcoma. A biopsy of the lesion revealed a granuloma with various stages of inflammation, no hemosiderin deposition, multinucleated giant cells, and foam cells containing fat, and was diagnosed with a foreign body granuloma. Special staining for microorganisms revealed no findings suggestive of infection. Because the patient was asymptomatic, no treatment was administered. Contrast-enhanced CT at 24 months postoperatively showed shrinkage of the mass, with no change in size noted at 48 months postoperatively. This report highlights a foreign body granuloma that mimicked malignant tumors. Extravascular migration of the NBCA-lipiodol mixture casts likely contributed to granuloma formation. Radiologists should consider foreign body granulomas after embolization using NBCA into the abdominal arteries.
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  • 文章类型: Case Reports
    肉芽肿性皮炎是在皮肤中或在各种表现中系统性地看到的常见组织反应模式。肉芽肿性皮炎可以细分为感染性和非感染性类别。本文重点介绍一名非感染性肉芽肿性皮炎患者,随访多年。既往史包括双侧全肩关节置换术并发假体关节感染。在其早期阶段,腋窝皮疹疼痛,并有许多充满液体的水泡。最终,皮疹的组织学认为病变非感染性,主要是由于炎症过程。具体来说,该患者使用了电离辐射。肉芽肿过程的类别很广泛,有许多亚型。非感染性肉芽肿过程的其他治疗选择可能包括皮质类固醇,光疗,和γ-干扰素注射.肉芽肿过程的差异是广泛的,治疗应根据具体情况决定。
    Granulomatous dermatitis is a common tissue reaction pattern seen in the skin or systematically in various presentations. Granulomatous dermatitis can be subclassified into infectious and non-infectious categories. This article focuses on a patient with non-infectious granulomatous dermatitis followed for many years. Past medical history included bilateral total shoulder arthroplasty complicated by prosthetic joint infections. In its early stages, the axillary rash was painful and had many fluid-filled blisters. Ultimately, the histology of the rash deemed the lesion non-infectious and mostly due to an inflammatory process. Specifically, ionizing radiation was used for this patient. The category of granulomatous processes is broad and there are many subtypes. Other treatment options for non-infectious granulomatous processes may include corticosteroids, phototherapy, and interferon-gamma injections. The differential for granulomatous processes is extensive and treatment should be decided on a case-by-case basis.
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  • 文章类型: Case Reports
    背景:眼部结核是结核病的一种相对罕见的肺外表现。这种威胁视力的疾病的诊断极具挑战性,特别是因为它可以模仿其他疾病。我们报告了一例结核性睫状体肉芽肿,最初被诊断为大疱性视网膜脱离。
    方法:一名52岁女性,左眼出现大疱性视网膜脱离,超声生物显微镜(UBM)证实存在睫状体肉芽肿性炎症。T-SPOT呈阳性,纯化蛋白衍生物(PPD)检测结果强阳性(直径20mm)。口服抗结核方案联合泼尼松后,视网膜逐渐重新附着,睫状体肉芽肿的大小明显缩小,患者的视力明显改善。
    结论:结核性睫状体肉芽肿可引起大疱性渗出性视网膜脱离,可诊断为UBM。早期全程抗结核治疗(ATT)联合糖皮质激素治疗可改善患者预后。
    BACKGROUND: Ocular tuberculosis is a relatively rare extrapulmonary manifestation of tuberculosis. This vision-threatening disease is extremely challenging to diagnose, particularly because it can mimic other diseases. We report a case of tuberculous ciliary body granuloma initially diagnosed as bullous retinal detachment.
    METHODS: A 52-year-old female presented with bullous retinal detachment in her left eye, and ultrasound biomicroscopy (UBM) verified the presence of a lesion with ciliary body granulomatous inflammation. The T-SPOT was positive, and the purified protein derivative (PPD) test was strongly positive (diameter of 20 mm). Following the administration of oral anti-tuberculosis regimen combined with prednisone, the retina gradually became reattached, the ciliary body granuloma became significantly reduced in size, and the visual acuity of the patient noticeably improved.
    CONCLUSIONS: Tuberculous ciliary body granulomas can cause bullous exudative retinal detachment and can be diagnosed with UBM. Early and full-course anti-tuberculosis treatment (ATT) combined with corticosteroid therapy can improve the patient prognosis.
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  • 文章类型: Journal Article
    肉芽肿性多血管炎是一种进行性坏死性肉芽肿性血管炎,病变主要累及小动脉、小静脉及毛细血管,属于自身免疫性疾病。普通型裂褶菌为机会性致病真菌,主要经呼吸道传播,常继发于其他基础病。本文介绍1例19岁男性患者,因咳嗽、发热、肺部占位就诊确诊为肉芽肿性多血管炎继发普通型裂褶菌感染的病例,并对其相关组织病理学特征进行阐述和讨论,以加深病理医师对该病的认识。.
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  • 文章类型: Case Reports
    探讨中枢神经系统(CNS)累及儿童嗜酸性肉芽肿合并多血管炎(EGPA)的临床特点及治疗效果。
    一个因中枢神经系统受累而出现EGPA的儿童于2023年6月入院。回顾性分析其临床特点,并对相关文献进行了综述,对这一状况进行了全面概述。
    一个十岁的女孩,有反复咳嗽和哮喘病史,伴有外周血嗜酸性粒细胞增多八个月,住进了我们的医院.一入场,在她的手和脚上可见斑点丘疹,可听到双侧肺部啰音。实验室检查显示嗜酸性粒细胞(EOS)的比例超过白细胞的10%,抗中性粒细胞胞浆抗体(MPO-ANCA)阳性,免疫球蛋白G水平为15.80g/L,免疫球蛋白E水平大于2500.00IU/mL。影像学检查显示双肺以及鼻窦炎有多个斑片状和结节状高密度阴影。肺功能检查提示中度通气和弥散功能障碍。骨髓细胞学证实嗜酸性粒细胞比例显著增加。皮肤病理证实为白细胞碎裂性血管炎。住院期间,这孩子抽搐了。大脑的磁共振成像(MRI)扫描显示双侧大脑皮层有多个异常信号阴影,脑电图(EEG)显示癫痫波。甲基强的松龙冲击治疗联合环磷酰胺治疗后,她的咳嗽和哮喘缓解了,皮疹消失,没有任何进一步的抽搐。我们发现,以前仅报道了一名年轻的中枢神经系统受累的EGPA患者。先前报告的病例始于长期发烧,减肥,紫癜性皮疹.两名患者对糖皮质激素和环磷酰胺治疗反应良好,他们的临床症状显着改善,外周血嗜酸性粒细胞正常化。
    在儿童中诊断EGPA可能具有挑战性。当孩子受到EGPA的影响时,必须对中枢神经系统受累的迹象保持警惕.糖皮质激素和环磷酰胺治疗可有效控制儿童EGPA。
    UNASSIGNED: To explore the clinical characteristics and treatment outcomes of children with central nervous system (CNS) involvement in eosinophilic granulomatosis with polyangiitis (EGPA).
    UNASSIGNED: A child who presented with EGPA complicated by CNS involvement was admitted to our hospital in June 2023. The clinical features were analyzed retrospectively, and relevant literatures were reviewed to provide a comprehensive overview of this condition.
    UNASSIGNED: A ten-year-old girl, who had a history of recurrent cough and asthma accompanied by peripheral blood eosinophilia for eight months, was admitted to our hospital. On admission, spotted papules were visible on her hands and feet, bilateral pulmonary rales were audible. The laboratory examination revealed that the proportion of eosinophils (EOS) exceeded 10% of white blood cells, the anti-neutrophil cytoplasmic antibody (MPO-ANCA) was positive, the immunoglobulin G level was 15.80g/L, and the immunoglobulin E level was greater than 2500.00IU/mL. The imaging examination showed multiple patchy and nodular high-density shadows in both lungs as well as sinusitis. Pulmonary function tests indicated moderate ventilation and diffusion dysfunction. Bone marrow cytology demonstrated a significant increase in the proportion of eosinophils. Skin pathology confirmed leukocytoclastic vasculitis. During the hospitalization, the child had a convulsion. The magnetic resonance imaging (MRI) scan of the brain showed multiple abnormal signal shadows in the bilateral cerebral cortex and the electroencephalogram (EEG) showed epileptic waves. Following the administration of methylprednisolone pulse therapy in combination with cyclophosphamide treatment, her cough and asthma resolved, the skin rash disappeared without any further convulsions. We found that only a young EGPA patient with CNS involvement had been previously reported. The previously reported case began with long-term fever, weight loss, and purpuric rash. Both patients responded well to treatment with glucocorticoids and cyclophosphamide, experiencing significant improvement in their clinical symptoms and normalization of their peripheral blood eosinophils.
    UNASSIGNED: The diagnosis of EGPA in children can be challenging. When a child is affected by EGPA, it is essential to remain vigilant for signs of CNS involvement. The treatment with glucocorticoids and cyclophosphamide is effective in managing EGPA in children.
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  • 文章类型: Case Reports
    食物颗粒经常在口腔中的不同部位受到影响。口腔脉冲肉芽肿是由于在口腔组织中植入植物或植物来源的食物颗粒而发展的。病变可能是中央或周围。它具有不同的组织学特征,可能与包括寄生虫感染在内的许多其他病变相混淆。受影响的植物性物质很少与多发性骨髓瘤相关,细菌性和真菌性骨髓炎,和鳞状细胞癌,以前从未在文献中报道过。因此,在本案系列中,我们已经讨论了以上三种罕见的关联以及与根尖周围肉芽肿和根尖周围囊肿相关的脉冲肉芽肿。本文旨在探讨临床,组织学,和两极分化的微观特征的病例和理论证实了病因和一般的鉴别诊断。
    Food particles get frequently impacted in the oral cavity in varying sites. Oral pulse granuloma develops as a consequence of implantation of food particles of plant or vegetable origin in oral tissues. The lesion might be central or peripheral. It has a differing histological picture and may be confused with many other lesions including parasitic infection. Impacted vegetable matter is rarely associated with multiple myeloma, bacterial and fungal osteomyelitis, and squamous cell carcinoma and has never been reported in the literature before. Thus, in the present case series, we have discussed about the above three rare associations along with pulse granulomas associated with periapical granuloma and periapical cyst. The paper aims to discuss the clinical, histological, and polarising microscopic features of the cases and theories substantiating aetiology and in general about the differential diagnosis.
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  • 文章类型: Letter
    暂无摘要。
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