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Ocular adnexal histiocytic disorders, including adult-onset asthma and periocular xanthogranuloma (AAPOX), necrobiotic xanthogranuloma and Erdheim-Chester disease, have common histological characteristics, such as the presence of foamy histiocytes (xanthoma cells) and Touton giant cells.1–3 Additionally, AAPOX presents lymphoid aggregation with prominent T cells and other histological variations with prominent B cells and plasma cells.2–5 AAPOX is also associated with unique systemic disorders, including adult-onset asthma, systemic lymphadenopathy, salivary gland enlargement and elevated serum levels of IgG.1–6 The causal relationships between the systemic characteristics and histological variations have not been determined.
To investigate the relationship between the systemic and histological associations of AAPOX in more detail, we studied four patients with AAPOX. We compared the clinicopathological features of AAPOX with those of ocular adnexal IgG4-related lymphoplasmacytic infiltrative disorder (IgG4-related ophthalmic disease).7
Procedures used in this study conformed to the tenets of the Declaration of Helsinki, and were approved by the Ethics Committee at Nagoya Medical Center, Nagoya, Japan.
A 38-year-old man presented with bilateral swollen eyelids that had gradually worsened over 9 years (figure 1). He had had autoimmune pancreatitis 10 years earlier and asthma 4 years earlier. He had received oral prednisolone, and 5 mg of oral prednisolone was given every other day before our initial examination. The laboratory data showed that the serum level of IgG4 was 525 mg/dL (reference range, 4.8–105 mg/dL). CT imaging showed that the lesions had bilateral periocular and …
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