PDF(Pubmed)
Abstract：
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.
摘要：
免疫球蛋白G4相关疾病（IgG4-RD）是一种与纤维炎症相关的自身免疫性疾病，可影响多个器官。IgG4-RD的标志性组织病理学发现包括IgG4阳性浆细胞的淋巴细胞浸润，storiform纤维化，和闭塞性静脉炎.然而,对IgG4-RD的中枢神经系统受累知之甚少。肥厚性硬脑膜炎（HP）最近被报道为IgG4-RD的表现，以前可能已经在很大一部分特发性病例中得到证实。在这里，我们报道了一例罕见的病例，一名63岁的男性，表现为模仿脑肿瘤的头皮肿块。手术后诊断为IgG4相关HP（IgG4-RP）。这种情况表明，在孤立的头皮肿块患者中，对IgG4-RP的可能性的认识，即使没有全身症状，至关重要。谨慎的历史结合，评估血清IgG4水平和影像学作为初步检查，接着是组织活检,对IgG4-RP的鉴别诊断很重要，恶性肿瘤，和其他传染病。
