Yeo Jina, Yee Gi Taek, Seo Jaedeok, Seo Mi Ryoung, Baek Han Joo, Choi Hyo-Jin
Division of Rheumatology, Department of Internal Medicine, Gachon University Gil Medical Center, Incheon, Korea.
Department of Neurosurgery, Gachon University Gil Medical Center, Incheon, Korea.
J Rheum Dis. 2024 Jan 1;31(1):54-58. doi: 10.4078/jrd.2023.0023. Epub 2023 Jul 28.
Immunoglobulin G4-related disease (IgG4-RD) is an autoimmune disorder associated with fibroinflammatory conditions that can affect multiple organs. Hallmark histopathological findings of IgG4-RD include lymphocytic infiltration of IgG4-positive plasma cells, storiform fibrosis, and obliterative phlebitis. However, little is known about central nervous system involvement of IgG4-RD. Hypertrophic pachymeningitis (HP) has recently been reported as a manifestation of IgG4-RD, which may have previously been demonstrated in a significant percentage of idiopathic cases. Herein, we report a rare case of a 63-year-old male who presented with a scalp mass that mimicked a brain tumor. He was diagnosed with IgG4-related HP (IgG4-RP) after surgery. This case suggests that awareness of a possibility of IgG4-RP in patients with isolated scalp masses, even in the absence of systemic symptoms, is crucial. A combination of careful history taking, evaluation of serum IgG4-levels and imaging as an initial work-up, followed by tissue biopsy, is important for the differential diagnosis of IgG4-RP, malignancy, and other infectious diseases.
免疫球蛋白G4相关疾病（IgG4-RD）是一种与纤维炎症性疾病相关的自身免疫性疾病，可累及多个器官。IgG4-RD的标志性组织病理学表现包括IgG4阳性浆细胞的淋巴细胞浸润、席纹状纤维化和闭塞性静脉炎。然而，关于IgG4-RD累及中枢神经系统的情况知之甚少。肥厚性硬脑膜炎（HP）最近被报道为IgG4-RD的一种表现，在以前相当比例的特发性病例中可能已有显示。在此，我们报告一例罕见病例，一名63岁男性，表现为类似脑肿瘤的头皮肿块。术后他被诊断为IgG4相关的HP（IgG4-RP）。该病例表明，即使在没有全身症状的情况下，对于孤立性头皮肿块患者，认识到IgG4-RP的可能性至关重要。仔细询问病史、评估血清IgG4水平并结合影像学作为初始检查，随后进行组织活检，对于IgG4-RP、恶性肿瘤和其他感染性疾病的鉴别诊断很重要。
