AI Article Synopsis
- The study reports a case of Adult-Onset Asthma with Periocular Xanthogranulomas (AAPOX) linked to systemic IgG4-related disease (IgG4-RD) in a 57-year-old man.
- The patient experienced periorbital swelling, and a biopsy revealed specific histological features such as foamy histiocytes and increased IgG4 positive plasma cells.
- The findings indicate that AAPOX may be related to systemic IgG4-RD, highlighting the need for further investigation into this condition.
Article Abstract
Purpose: The aim of this study was to report a case of Adult-Onset Asthma with Periocular Xanthogranulomas (AAPOX) associated with systemic IgG4-related disease (IgG4-RD).
Observations: A 57-year-old man presented with bilateral periorbital swelling for 1 year. Histopathology of a left orbital biopsy showed fibro vascular connective tissue inundated with foamy, lipid-laden histiocytes and touton giant cells with lymphocytic inflammation. Additional stains revealed CD68 positivity, and S100 negativity. The IgG and IgG4 stained slides showed increased IgG4 positive plasma cells but did not meet the criteria for IgG4-related orbital disease. His IgG4 serology was elevated, and IgG4 staining of his tissue previously diagnosed as autoimmune sclerosing pancreatitis was found to meet the criteria for IgG4-RD.
Conclusions And Importance: AAPOX can be associated with systemic IgG4-RD.
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Source |
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| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4852489 | PMC |
| http://dx.doi.org/10.1016/j.ajoc.2016.03.006 | DOI Listing |
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