Concomitant adult onset xanthogranuloma and IgG4-related orbital disease: a rare occurrence.

Concomitant adult onset xanthogranuloma and IgG4-related orbital disease: a rare occurrence.
Orbit. 2020 Aug 26;:1-4
Authors: Andron AA, Nair AG, Della Rocca D, Della Rocca RC, Reddy HS
Abstract
Adult onset xanthogranulomatous disease is a rare orbital disease. IgG4-related disease is a systemic disease that can often manifest in the orbit. In this communication, we present the case of a patient with a xanthelasma-like lesion on the upper eyelid, and an enlarged lacrimal gland, which on biopsy was diagnosed as an orbital xanthogranuloma. Detailed serological workup showed that the patient was found to have elevated serum IgG4 levels. The orbital specimen was re-stained and found to be positive for IgG4. The patient was treated with oral steroids with partial resolution of the lesion. This is an interesting case of both histopathological adult onset xanthogranuloma (AOX) and IgG4-related orbital disease (IgG4-ROD). The early evidence suggests that the diagnosis of one of these disorders should point the physician to investigate for the presence of the other, especially if xanthogranulomatous disease is diagnosed first.
PMID: 32847452 [PubMed - as supplied by publisher]

Source: Orbit - August 25, 2020 Category: Opthalmology Authors: Andron AA, Nair AG, Della Rocca D, Della Rocca RC, Reddy HS Tags: Orbit Source Type: research