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KMID : 1084220240310010054
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Journal of Rheumatic Diseases
2024 Volume.31 No. 1 p.54 ~ p.58
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Immunoglobulin G4-related hypertrophic pachymeningitis with an isolated scalp mass mimicking a brain tumor: a case report and literature review
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Yeo Ji-Na
Yee Gi-Taek
Seo Jae-Deok
Seo Mi-Ryoung
Baek Han-Joo
Choi Hyo-Jin
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Abstract
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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.
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KEYWORD
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Brain neoplasms, Hypertrophic pachymeningitis, Immunoglobulin G4-related disease, Immunoglobulin G4-related pachymenigitis
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