A 28-year-old woman presented with progressive proptosis of the left eye. She had a history of familial thrombocytopenia and Hashimoto thyroiditis. CT of the orbits demonstrated a bilateral diffuse intraconal and extraconal infiltration. Biopsy from the left intraconal area revealed the typical histopathology of xanthogranuloma with a mixture of foamy histiocytes, Touton giant cells, and eosinophils. Systemic examination revealed a mediastinal and retroperitoneal infiltration with a focal lesion in the left kidney. A biopsy of the retroperitoneal area showed histopathology identical to that of the orbital lesion. A review of the literature indicated that the association between non-Langerhans histocytoses and immunologic dysfunctions is not uncommon. We hypothesize that Erdheim-Chester disease may be linked to an abnormal interaction between T-lymphocytes and macrophages similarly to the macrophage activation syndromes.
|Original language||English (US)|
|Number of pages||3|
|Journal||Ophthalmic plastic and reconstructive surgery|
|State||Published - Jan 1 2006|
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