Adult ocular adnexal xanthogranulomatous disease associated with immunoglobulin G4-related disease: an unusual association

Abstract

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.

Keywords

• IgG4-related disease
• adult-onset xanthogranuloma
• orbit