The Spectrum of Cutaneous Granulomatous Inflammation and Detection of Rubella Virus in Skin Biopsies of Patients With Common Variable Immune Deficiency.

IF 1.6 4区 医学 Q3 DERMATOLOGY
Anne L King, Emma F Johnson, Afsaneh Alavi, Shruti Agrawal, Olayemi Sokumbi, Ludmila Perelygina, Laura Yorke, Suzanne Beard, Carilyn N Wieland
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Abstract

Background: There is a known association between common variable immunodeficiency (CVID) and granulomas in multiple organ systems, including the skin, lung, liver, and spleen. Rubella virus has also been detected within cutaneous granulomas in both immunocompetent and immunocompromised hosts. We present a retrospective case series of patients with CVID and granulomatous skin disease and describe the spectrum of clinical and histopathologic features, including the status of rubella virus in the cutaneous granulomas.

Methods: We retrospectively reviewed the clinical and histopathological characteristics of patients diagnosed with CVID at our institution, with cutaneous findings and skin biopsies available for review between 1990 and 2023, demonstrating granulomatous inflammation.

Results: Eight patients met the inclusion criteria. The most common histopathologic pattern was palisaded granulomatous inflammation, seen in five of eight cases. Three cases showed strictly sarcoidal granulomas. Background inflammation was peri-granulomatous (8/8) and the predominant background inflammatory cell type was lymphocytic (6/8). Rubella virus testing was performed for seven of eight cases and found to be positive in one case.

Conclusion: Cutaneous granulomatous disease in CVID can present with a spectrum of clinical morphologies, granulomatous patterns, and variable rubella virus persistence. Dermatopathologists should be aware of the spectrum of findings when considering cutaneous CVID-related granulomatous disease in the differential diagnosis.

常见变异性免疫缺陷患者皮肤肉芽肿性炎症的范围及皮肤活检中风疹病毒的检测。
背景:众所周知,常见变异性免疫缺陷症(CVID)与多个器官系统(包括皮肤、肺、肝脏和脾脏)的肉芽肿之间存在关联。在免疫功能正常和免疫功能低下的宿主的皮肤肉芽肿中也检测到了风疹病毒。我们对 CVID 和肉芽肿性皮肤病患者进行了回顾性病例系列研究,描述了临床和组织病理学特征,包括风疹病毒在皮肤肉芽肿中的状态:我们回顾性分析了1990年至2023年间本院确诊的CVID患者的临床和组织病理学特征,这些患者的皮肤检查结果和皮肤活检结果均显示有肉芽肿性炎症:结果:8 名患者符合纳入标准。最常见的组织病理学模式是苍白肉芽肿性炎症,8 例中有 5 例。三例病例表现为严格的肉芽肿。背景炎症为肉芽肿周围炎(8/8),主要的背景炎症细胞类型为淋巴细胞(6/8)。8 例病例中有 7 例进行了风疹病毒检测,其中 1 例呈阳性:结论:CVID 患者的皮肤肉芽肿性疾病可表现为多种临床形态、肉芽肿模式和可变的风疹病毒持续存在。皮肤病理学家在鉴别诊断中考虑与 CVID 相关的皮肤肉芽肿病时,应了解其研究结果的范围。
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来源期刊
CiteScore
3.20
自引率
5.90%
发文量
174
审稿时长
3-8 weeks
期刊介绍: Journal of Cutaneous Pathology publishes manuscripts broadly relevant to diseases of the skin and mucosae, with the aims of advancing scientific knowledge regarding dermatopathology and enhancing the communication between clinical practitioners and research scientists. Original scientific manuscripts on diagnostic and experimental cutaneous pathology are especially desirable. Timely, pertinent review articles also will be given high priority. Manuscripts based on light, fluorescence, and electron microscopy, histochemistry, immunology, molecular biology, and genetics, as well as allied sciences, are all welcome, provided their principal focus is on cutaneous pathology. Publication time will be kept as short as possible, ensuring that articles will be quickly available to all interested in this speciality.
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