Granulomatous

肉芽肿
  • 文章类型: Case Reports
    一位35岁的先生主诉双侧眼痛来到眼科门诊部,发红和畏光自三周以来,具有相似的既往史。该患者是自两年以来诊断为全身性结节病的病例,他已经接受了包括口服免疫抑制剂在内的皮肤和神经系统疾病的治疗,类固醇,抗惊厥药和多种维生素。在检查中,右眼的最佳矫正视力为6/18,左眼为6/12。裂隙灯和眼底检查,患者双眼均出现前葡萄膜炎和后葡萄膜炎的征象,右眼比左眼多。使用局部皮质类固醇和β受体阻滞剂开始治疗,患者在医疗管理后有所改善。
    A 35-year-old gentleman came to the ophthalmology outpatient department with complaints of bilateral ocular pain, redness and photophobia since three weeks with similar prior history. The patient was a diagnosed case of systemic sarcoidosis since two years with pulmonary, dermatological and neurological involvement for which he was already on treatment which included oral immunosuppressants, steroids, anticonvulsants and multivitamins. On examination, the best corrected visual acuity was 6/18 in the right eye and 6/12 in the left eye. On slit lamp and fundus examination, the patient showed signs of anterior and posterior uveitis in both eyes, the right eye more than the left eye. Treatment was initiated with topical corticosteroids and beta blockers and the patient improved following medical management.
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  • 文章类型: Journal Article
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  • 文章类型: Journal Article
    背景:小儿肉芽肿性葡萄膜炎(PGU)很少见。此外,缺乏意识通常会导致诊断延迟和视力不良。确定根本原因并决定如何最好地治疗每位患者具有挑战性。
    目的:为了评估人口统计学,病因学,并发症,治疗,小儿非感染性肉芽肿性葡萄膜炎的视觉预后。
    方法:从儿科风湿病科招募的16岁前儿童非感染性PGU的回顾性图表回顾,Bicettre医院,法国,从2001年到2023年。
    结果:我们包括50例患者,其中90只受影响的眼睛:29例特发性葡萄膜炎,15患有结节病,5患有幼年特发性关节炎,还有一个患有Vogt-Koyanagi-Harada病.诊断时的中位年龄为9.8岁(范围7.2-12.5)。性别比M/F为0.52。PGU最常见的特征是:全葡萄膜炎(56%),双边(84%),慢性(84%)。结节病是特发性疾病后最常见的诊断,特别是在存在淋巴细胞减少和高丙种球蛋白血症的情况下。12%的病例存在葡萄膜脑膜炎。诊断后,90眼中有68眼(76%)出现眼部并发症,尤其是在全葡萄膜炎病例中。最常用的治疗是全身性皮质类固醇(72%)和甲氨蝶呤(80%)。在最后一次随访时,23%的眼睛处于缓解状态,68%无活性,4%保持活性。中位随访时间为5.8年。
    结论:我们报告了PGU的最大队列。PGU多为特发性,并发症发生率高。结节病和特发性全葡萄膜炎是严重的疾病,在诊断时应开始疾病修饰治疗以改善管理。
    BACKGROUND: Paediatric granulomatous uveitis (PGU) is rare. In addition, lack of awareness often leads to delayed diagnosis and poor visual outcome. Identifying the underlying cause and deciding how best to treat each patient is challenging.
    OBJECTIVE: To evaluate the demographics, aetiologies, complications, treatments, and visual prognosis of paediatric non-infectious granulomatous uveitis.
    METHODS: Retrospective chart review of non-infectious PGU occurring in children before the age of 16 years recruited from the Paediatric Rheumatology Unit, Bicêtre Hospital, France, from 2001 to 2023.
    RESULTS: We included 50 patients with 90 affected eyes: 29 with idiopathic uveitis, 15 with sarcoidosis, 5 with juvenile idiopathic arthritis, and one with Vogt-Koyanagi-Harada disease. Median age at diagnosis was 9.8 years (range 7.2-12.5). The sex-ratio M/F was 0.52. The most common features of PGU were: panuveitis (56%), bilateral (84%), and chronic (84%). Sarcoidosis was the most frequent diagnosis after idiopathic disease, particularly in the presence of lymphopenia and hypergammaglobulinemia. Uveomeningitis was present in 12% of cases. Upon diagnosis, ocular complications were present in 68 of 90 eyes (76%) particularly in cases of panuveitis. The most commonly used treatments were systemic corticosteroids (72%) and methotrexate (80%). Twenty-three percent of eyes were in remission at last follow-up, 68% were inactive and 4% remained active. The median duration of follow-up was 5.8 years.
    CONCLUSIONS: We report the largest cohort of PGU. PGU were mostly idiopathic and had a high rate of complications. Sarcoid and idiopathic panuveitis are serious illnesses in which disease-modifying therapy should be initiated at diagnosis to improve management.
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  • 文章类型: Case Reports
    肉芽肿性炎症是慢性炎症反应的独特变体。口腔面部组织可能会受到各种肉芽肿疾病的影响。病变范围包括感染,免疫学,和反应,异物肉芽肿.众所周知,结核病(TB)是一种慢性传染病,可以影响身体的任何部位,包括嘴巴。它可能涉及口腔中的舌头,并具有相当独特的特征和形式。因此,虽然不常见,口腔病变对早期发现和治疗原发性结核病至关重要.我们讨论了牙龈TB的可能实例,该实例表现为牙龈肿大。患者接受测试剂量的抗结核治疗一个月。抗结核治疗在一个月的治疗显示进展后的五个月内完成。这份针对牙医的病例报告强调了在各种类型的牙龈扩张的鉴别诊断中考虑结核病的重要性。
    Granulomatous inflammation is a distinctive variant of the chronic inflammatory response. The orofacial tissues may be affected by a wide range of granulomatous diseases. The lesions range from infections, immunological, and reactive, to foreign body granulomas. As is common knowledge, tuberculosis (TB) is a chronic infectious disease that can affect any region of the body, including the mouth. It may involve the tongue in the mouth and have quite peculiar features and forms. Therefore, while uncommon, oral lesions are crucial for the early detection and treatment of primary TB. We discuss a possible instance of gingival TB that manifested as an enlarged gingiva. The patient received a test dose of antituberculous therapy for one month. The antituberculous therapy was completed for the following five months after one month of treatment showed progress. This case report for dentists emphasizes how crucial it is to consider TB in the differential diagnosis of various types of gingival enlargements.
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  • 文章类型: Case Reports
    特发性肉芽肿性乳腺炎并不常见,女性乳房的长期炎症状况。它的特征是疼痛的乳房肿块的发展,其大小逐渐增加。这种情况是良性的,原因不明。它主要影响有怀孕和母乳喂养史的育龄妇女。特发性肉芽肿性乳腺炎的主要特征是乳房中存在慢性炎症。
    方法:我们报告了一名36岁女性,有妊娠和哺乳史,右侧乳房疼痛和肿胀。体格检查显示肿块,乳头发红和缩回。成像显示局部密度和低回声区域与收集,提示肉芽肿性乳腺炎.活检证实了诊断。在局部麻醉下通过小切口引流脓肿。用皮质类固醇治疗导致显着改善,一个月后完成决议。
    必须对潜在原因进行全面评估,以确认特发性肉芽肿性乳腺炎的诊断。组织学上,它的特点是主要存在中性粒细胞和不存在干酪样坏死。治疗仍然存在争议,最近的文献支持保守治疗与类固醇和免疫抑制治疗的疗效,对复杂和难治性病例进行手术切除。
    结论:特发性肉芽肿性乳腺炎是一种罕见的乳腺疾病,其确切原因和推荐的治疗方法尚不明确。重要的是要考虑到处于生育期的女性的这种情况。
    UNASSIGNED: Idiopathic granulomatous mastitis is an uncommon, long-lasting inflammatory condition of the female breast. It is characterized by the development of a painful breast mass that gradually increases in size. This condition is benign and its cause is unknown. It primarily affects women of childbearing age who have a history of pregnancy and breastfeeding. The main feature of idiopathic granulomatous mastitis is the presence of chronic inflammation in the breast.
    METHODS: We report a 36-year-old woman with a history of pregnancy and breastfeeding presented with pain and swelling of the right breast. Physical examination revealed a mass with redness and retraction of the nipple. Imaging revealed localized density and hypoechoic areas with collection, suggesting granulomatous mastitis. A biopsy confirmed the diagnosis. The abscess was drained through a small incision performed under local anesthesia. Treatment with corticosteroids resulted in significant improvement, with complete resolution after one month.
    UNASSIGNED: A comprehensive evaluation of potential causes is necessary to confirm the diagnosis of idiopathic granulomatous mastitis. Histologically, it is distinguished by the predominant presence of neutrophils and the absence of caseous necrosis. Treatment remains controversial, with recent literature supporting the efficacy of conservative management with steroid and immunosuppressive therapy, leaving surgical excision for complicated and refractory cases.
    CONCLUSIONS: Idiopathic granulomatous mastitis is an uncommon breast condition where the exact causes and recommended treatment approaches are not well-defined. It is important to consider this condition in women who are in their reproductive years.
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  • 文章类型: Case Reports
    背景:鼻鼻孢子虫病是指一种罕见的慢性肉芽肿性疾病,由雪柏鼻虫引起。它影响鼻咽等部位的粘膜,结膜和腭。内翻性乳头状瘤相对罕见,是鼻腔良性上皮性肿瘤,具有局部侵袭性,表现出复发倾向和恶变。这两个实体在我们的环境中都非常罕见,这可能是坦桑尼亚第一个有记录的案例。
    方法:患者为一名7岁男孩,有1年左侧鼻塞和间歇性鼻出血6个月的病史。他没有脸颊肿胀的病史,疼痛或麻木,牙齿脱落或松动或牙槽嵴丰满。没有眼科检查,耳科或神经科投诉报告。完成了鼻肿块的内窥镜切除,组织病理学分析证实了鼻孢子虫病和内翻性乳头状瘤并存。术后,患者口服氨苯砜,剂量为50mg/d,持续6个月,随访6个月后未发现残留疾病复发.
    患者接受鼻内镜切除鼻块,组织病理学分析证实鼻孢子虫病和内翻性乳头状瘤并存。术后,患者口服氨苯砜,剂量为50mg/d,持续6个月,随访6个月后未发现残留疾病复发.
    结论:鼻鼻孢子虫病和内翻性乳头状瘤病变可能与常规鼻息肉相似,因此对于临床医生和病理学家来说,在处理鼻腔肿块的患者时,即使是非地方区域也要高度怀疑。
    BACKGROUND: Nasal rhinosporidiosis refers to a rare chronic granulomatous disease caused by Rhinosporidium seeberi. It affects the mucous membrane of sites such as nasopharynx, conjunctiva and palate. Inverted papillomas are relatively rare and are benign epithelial tumors of the nasal cavity that are locally aggressive, exhibit recurrence tendency and malignant transformation. Both entities are very rare in our setting and this is perhaps the first documented case in Tanzania.
    METHODS: The patient was a 7-year old boy with a 1-year history of left-sided nasal obstruction and intermittent epistaxis for 6 months. He had no history of cheek swelling, pain or numbness, loss or loosening of teeth or alveolar ridge fullness. There were no ophthalmological, otological or neurological complaints reported. Endoscopic excision of the nasal mass was done and histopathological analysis confirmed co-existence of rhinosporidiosis and inverted papilloma. Postoperatively, the patient was kept on oral dapsone at a dose of 50 mg/day for 6-months and with no residual disease recurrence noted after 6-months follow up.
    UNASSIGNED: The patient underwent endoscopic excision of the nasal mass and histopathological analysis confirmed co-existence of rhinosporidiosis and inverted papilloma. Postoperatively, the patient was kept on oral dapsone at a dose of 50 mg/day for 6-months and with no residual disease recurrence noted after 6-months of follow up.
    CONCLUSIONS: Nasal rhinosporidiosis and inverted papilloma lesions may resemble the routinely encountered nasal polyps thus important for both clinicians and pathologists to have a high index of suspicion when managing patients with nasal masses even from non-endemic areas.
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  • 文章类型: Case Reports
    膀胱尿路上皮癌仍然是一种具有挑战性的疾病。膀胱内滴注BCG在预防复发方面表现出巨大的功效。BCG相关的坏死性肉芽肿性附睾-睾丸炎很少见,并且以前与前列腺腺癌的近距离放射治疗无关。我们假设先前的近距离放射治疗对verumontanum有有害影响,可能导致BCG颗粒的逆行传播,导致肉芽肿性附睾-睾丸炎。这是第一例与BCG相关的坏死性肉芽肿性附睾睾丸炎在前列腺腺癌近距离放射治疗后的患者状态报告。
    Urothelial carcinoma of the bladder remains a challenging disease to treat. Intravesical instillation of BCG has demonstrated tremendous efficacy in preventing recurrence. BCG related necrotizing granulomatous epididymo-orchitis is rare and has not been previously linked to brachytherapy for adenocarcinoma of the prostate. We hypothesize that prior brachytherapy has a deleterious effect on the verumontanum that can result in retrograde transmission of BCG particles leading to granulomatous epididymo-orchitis. This is the first case report of necrotizing granulomatous epididymo-orchitis related to BCG in a patient status post brachytherapy for adenocarcinoma of the prostate.
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  • 文章类型: Journal Article
    背景:间质真菌病(IMF)是一种罕见的真菌病(MF)亚型,其特征是非典型淋巴细胞浸润胶原蛋白束之间的网状真皮,具有有限的表皮性和可变的肉芽肿特征。
    方法:回顾性单机构回顾了31例IMF的临床特征,病程和病理特征。
    结果:我们的队列主要是男性(19;61%,M:F1.6:1),诊断时的平均年龄为43岁(范围11-85),诊断前7年的平均体征/症状持续时间,和6年平均随访时间。临床上,患者通常表现出对称性不明确的斑块/斑块,涉及皮肤间区域,伴有黄褐色色素沉着和毛囊性丘疹,起皱,和脱发。7例患者出现淋巴结肿大。在最近的随访中,有15例(52%)患者处于接近或完全缓解的临床状态。23/24(96%)例T细胞受体基因重排呈阳性。组织病理学,非典型细胞是小介质,CD4+(29;94%)或很少有CD4+/CD8+(1;3%)淋巴细胞浸润网状真皮,胶原束增厚(27;87%),多核巨细胞(12;39%),并且经常沿着附件追踪,具有微妙的毛色性(12/20;60%)。
    结论:我们的研究表明,IMF是MF的惰性亚型,具有独特的特征,包括常见的肉芽肿和轻微的卵泡受累,导致脱发。
    BACKGROUND: Interstitial mycosis fungoides (IMF) is a rare subtype of mycosis fungoides (MF) characterized by atypical lymphocytes infiltrating the reticular dermis between collagen bundles with limited epidermotropism and variable granulomatous features.
    METHODS: Retrospective single institution review of 31 cases of IMF including clinical characteristics, disease course and pathological features.
    RESULTS: Our cohort was predominately male (19; 61%, M:F 1.6:1) with a mean age at diagnosis of 43 years (range 11-85), mean signs/symptoms duration of 7 years prior to diagnosis, and 6 years mean follow-up duration. Clinically, patients often exhibited symmetric ill-defined patches/plaques involving intertriginous regions with tan-yellow hyperpigmentation and follicular-based papules, wrinkling, and alopecia. Lymphadenopathy was noted in seven patients. Fifteen (52%) patients were in near or complete clinical remission at the latest follow-up. T-cell receptor gene rearrangement was positive in 23/24 (96%) cases. Histopathologically, atypical cells were small-medium, CD4+ (29; 94%) or rarely CD4+/CD8+ (1; 3%) lymphocytes infiltrating the reticular dermis with thickened collagen bundles (27; 87%), multinucleated giant cells (12; 39%), and often tracing along adnexa with subtle folliculotropism (12/20; 60%).
    CONCLUSIONS: Our study demonstrates IMF is an indolent subtype of MF with distinct features, including frequent granulomatous and subtle follicular involvement resulting in alopecia.
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  • 文章类型: Journal Article
    目的:描述33只接受眼球摘除和眼部组织病理学检查的无菌肉芽肿性全葡萄膜炎综合征的临床和组织病理学特征。
    方法:回顾33例患者的病历和眼部组织病理学报告。纳入标准是摘除术结合特征性临床和组织病理学特征。
    结果:代表了13个品种(包括杂交品种)。葡萄膜炎是急性和暴发性的,继发性青光眼是常见的(n=27)。从初次就诊到摘除的间隔为99天(中位数为33天,5-605天)。眼球摘除的平均年龄为6.7岁。眼部体征最初为单侧(n=18)或双侧(n=15)。在最初接受单侧摘除的18/25例病例中,该疾病变为双侧疾病,导致9/18的摘除或安乐死(平均间隔168天)。59只眼中的7只眼在局部抗炎和全身免疫抑制治疗后具有良好的结果。从就诊到随访,没有一条狗有旅行史或相关的全身体征(平均619天,范围16-3012天)。组织病理学显示组织细胞和淋巴浆细胞性葡萄膜炎伴色素分散,在光学显微镜上没有发现感染因子。
    结论:据作者所知,这是英国首次报道犬的无菌肉芽肿性葡萄膜炎综合征.临床症状很严重,随着快速发展,并可能导致受影响的狗的双侧摘除或安乐死。似乎没有年龄或品种倾向,然而,在这方面需要进一步的研究。早期和积极的干预,局部和全身免疫抑制治疗,建议减少失明的风险,摘除,和安乐死。
    OBJECTIVE: To describe the clinical and histopathological features of a sterile granulomatous panuveitis syndrome in 33 dogs that underwent enucleation and ocular histopathology.
    METHODS: Retrospective review of the medical records and ocular histopathology reports of 33 cases. Inclusion criteria were enucleation in conjunction with characteristic clinical and histopathological features.
    RESULTS: Thirteen breeds were represented (including crossbreeds). Panuveitis was acute and fulminating, and secondary glaucoma was common (n = 27). Interval from initial presentation to enucleation was 99 days (median 33 days, range 5-605 days). The mean age at enucleation was 6.7 years. Ocular signs were initially unilateral (n = 18) or bilateral (n = 15). The disease became bilateral in 18/25 cases that initially underwent unilateral enucleation, resulting in enucleation or euthanasia in 9/18 (mean interval of 168 days). Seven out of 59 eyes had a good outcome following topical anti-inflammatory and systemic immunosuppressive therapy. None of the dogs had travel history nor relevant systemic signs from presentation to follow-up (mean 619 days, range 16-3012 days). Histopathology revealed histiocytic and lymphoplasmacytic panuveitis with pigment dispersion, and no infectious agents were identified on light microscopy.
    CONCLUSIONS: To the authors\' knowledge, this is the first report of a sterile granulomatous panuveitis syndrome in dogs in the UK. The clinical signs are severe, with rapid progression, and can result in bilateral enucleation or euthanasia in affected dogs. There does not appear to be an age or breed predisposition, however further research is necessary in this regard. Early and aggressive intervention, with both topical and systemic immunosuppressive therapy, is recommended to reduce the risk of blindness, enucleation, and euthanasia.
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  • 文章类型: Case Reports
    骨髓增生异常综合征(MDS)是一组由干细胞突变引起的克隆性造血肿瘤。它们具有转化为急性髓细胞性白血病的风险。MDS的皮肤表现,包括骨髓增生异常角化或MDS肿瘤细胞浸润,是罕见的,但与进展为高级别髓系肿瘤的风险增加显著相关。皮肤骨髓增生异常的临床和组织病理学鉴别诊断包括间质性肉芽肿性皮炎(IGD),与全身性疾病相关的反应性肉芽肿性皮炎(RGD),包括风湿性疾病,和血液恶性肿瘤如MDS。我们报告了一名MDS患者,该患者在临床和组织病理学上都伪装成IGD。
    Myelodysplastic syndromes (MDS) are a group of clonal hematopoietic neoplasms resulting from mutations in stem cells. They carry a risk of transformation to acute myeloid leukemia. Cutaneous manifestations of MDS, including myelodysplasia cutis or infiltration by MDS tumor cells, are rare, but significantly associated with increased risk of progression to high-grade myeloid tumors. The clinical and histopathologic differential diagnosis for myelodysplasia cutis includes interstitial granulomatous dermatitis (IGD), a reactive granulomatous dermatitis (RGD) associated with systemic diseases including rheumatologic diseases, and hematologic malignancy like MDS. We report a patient with MDS who presented with myelodysplasia cutis masquerading as IGD both in a clinical and histopathological manner.
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