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Erdheim-Chester disease: a bilateral orbital mass as an indication of systemic disease

1999; Taylor & Francis; Volume: 18; Issue: 2 Linguagem: Inglês

10.1076/orbi.18.2.99.2714

1744-5108

Shantha Amrith, C. H. Low, Elizabeth Cheah, Yew Oo Tan,

Autoimmune and Inflammatory Disorders

A case of bilateral orbital mass and xanthelasma of the eyelids is presented. Histology confirmed it to be a form of histiocytosis, possibly an Erdheim-Chester disease. This was further confirmed by the presence of a retroperitoneal mass and hydronephrosis, which resolved with treatment. A review of the literature on and pathological features of this rare fatal disease is presented.