Rare coexistence of adult-onset asthma with periocular xanthogranuloma and Immunoglobulin G4-related disease
Abstract
Abstract IgG4-related disease (IgG4-RD) is a systemic fibroinflammatory condition affecting multiple organs, including the orbit. Adult-onset asthma with periocular xanthogranuloma (AAPOX) is a rare subtype that may share histopathologic and immunologic features with IgG4-RD. We report a 61-year-old man with a history of asthma and pancreatitis who presented with recurrent bilateral eyelid swelling and ptosis. Elevated serum IgG4 levels and dense IgG4-positive plasma cell infiltration in orbital tissue supported the diagnosis of IgG4-RD with overlapping AAPOX features. The patient showed an initial response to corticosteroids, and early rituximab therapy contributed to sustained remission, despite a transient relapse during tapering. During follow-up, an incidental intramucosal carcinoma arising in a sigmoid colon polyp was detected. This case highlights the rare coexistence of AAPOX and IgG4-RD, emphasizing the importance of comprehensive systemic evaluation and malignancy screening in patients with atypical or relapsing presentations.
Keywords
References
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Details
Primary Language
English
Subjects
Clinical Sciences (Other)
Journal Section
Case Report
Authors
Meryem Altın Ekin
*
Türkiye
Semih Gülle
0000-0001-8679-2130
Türkiye
Banu Lebe
0000-0003-2520-2664
Türkiye
Early Pub Date
April 29, 2026
Publication Date
April 29, 2026
Submission Date
July 18, 2025
Acceptance Date
September 28, 2025
Published in Issue
Year 2026 Volume: 40 Number: 2