Immunoglobulin G4-related hypertrophic pachymeningitis with an isolated scalp mass mimicking a brain tumor: a case report and literature review
- Author:
Jina YEO
1
;
Gi Taek YEE
;
Jaedeok SEO
;
Mi Ryoung SEO
;
Han Joo BAEK
;
Hyo-Jin CHOI
Author Information
- Publication Type:Case Report
- From:Journal of Rheumatic Diseases 2024;31(1):54-58
- CountryRepublic of Korea
- Language:EN
- 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.
