Bahiana School of Medicine and Public Health, Salvador, Brazil.

Bahiana School of Medicine and Public Health, Salvador, Brazil.

Bahiana School of Medicine and Public Health, Salvador, Brazil.

Professor Edgard Santos University Hospital Complex, Federal University of Bahia, Brazil.

Bahiana School of Medicine and Public Health, Salvador; Professor Edgard Santos University Hospital Complex, Federal University of Bahia; Specialized Rheumatology Services, Bahia, Brazil.

Adult-onset xanthogranuloma (AOX) and immunoglobulin G4-related disease (IgG4-RD) are uncommon fibrosing conditions that may exhibit localized ocular manifestations and occasionally systemic symptoms. These conditions exhibit overlapping clinical and histological features, suggesting a potential correlation between them, although their exact relationship remains unclear. This paper presents the case of a black male patient exhibiting typical histological indications of both AOX and IgG4-RD. The patient responded positively to corticosteroid treatment.

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