King Anne L, Johnson Emma F, Alavi Afsaneh, Agrawal Shruti, Sokumbi Olayemi, Perelygina Ludmila, Yorke Laura, Beard Suzanne, Wieland Carilyn N
Department of Dermatology, Mayo Clinic, Rochester, Minnesota, USA.
Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota, USA.
J Cutan Pathol. 2025 Mar;52(3):220-226. doi: 10.1111/cup.14761. Epub 2024 Nov 26.
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.
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.
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.
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.
已知常见可变免疫缺陷(CVID)与多器官系统中的肉芽肿有关,包括皮肤、肺、肝和脾。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。我们报告了一组CVID和肉芽肿性皮肤病患者的回顾性病例系列,并描述了临床和组织病理学特征谱,包括皮肤肉芽肿中风疹病毒的状态。
我们回顾性地分析了在我院诊断为CVID的患者的临床和组织病理学特征,这些患者在1990年至2023年期间有皮肤表现且有皮肤活检可供复查,显示为肉芽肿性炎症。
8例患者符合纳入标准。最常见的组织病理学模式是栅栏状肉芽肿性炎症,8例中有5例出现。3例显示为严格的结节病样肉芽肿。背景炎症为肉芽肿周围性(8/8),主要的背景炎症细胞类型为淋巴细胞(6/8)。8例中有7例进行了风疹病毒检测,其中1例呈阳性。
CVID中的皮肤肉芽肿性疾病可表现出一系列临床形态、肉芽肿模式和不同的风疹病毒持续存在情况。皮肤病理学家在鉴别诊断中考虑与CVID相关的皮肤肉芽肿性疾病时应了解这些发现谱。