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A Report of Two Cases

Jerry A. Shields, MD; Zeynel A. Karcioglu, MD; Carol L. Shields, MD; Ralph C. Eagle, MD; Stephen Wong, MD

Arch Ophthalmol. 1991;109(6):850-854.

Abstract
• Erdheim-Chester disease is an idiopathic condition characterized by infiltration of the heart, lungs, retroperitoneum, bones, and other tissues by a fibrosing xanthogranulomatous process composed of xanthomatous histiocytes and Touton giant cells. This condition is often fatal, with death due to cardiomyopathy, severe lung disease, or chronic renal failure. Ocular findings with this potentially fatal disease are rare. We report the clinical and histopathologic findings in two cases of bilateral xanthelasmas and bilateral orbital infiltrates in association with Erdheim-Chester disease. The first patient was a 38-year-old man with cardiovascular and renal disease and severe retroperitoneal fibrosis. The massive orbital infiltration produced bilateral blindness. The second patient was a 77-year-old man with severe cardiovascular disease and retroperitoneal fibrosis. The diagnosis was confirmed in both patients with retroperitoneal and orbital biopsies. Both patients had the unusual occurrence of bilateral xanthelasmas with bilateral, diffuse orbital masses, eye findings that should alert the clinician to the possibility of this serious systemic disease.

Author Affiliations
From the Ocular Oncology Service (Drs J. Shields and C. Shields) and the Department of Pathology (Dr Eagle), Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pa; the Department of Ophthalmology, Tulane University Medical Center, New Orleans, La (Dr Karcioglu); and the Department of Ophthalmology, Temple University Hospital, Philadelphia (Dr Wong).

Footnotes
Accepted for publication March 12, 1991.

Case 1 was presented at the 1987 meeting of the Theobald Society, Captiva Island, Fla, May 1, 1987 (by Dr Karcioglu), and Case 2 was presented at the 1989 meeting of the Eastern Ophthalmic Pathology Society, Hilton Head, SC, October 7, 1989 (by Dr J. Shields). Both cases were presented at the International Society of Ophthalmic Pathology, New Orleans, La, October 28, 1989 (Dr J. Shields).

Reprint requests to the Ocular Oncology Service, Wills Eye Hospital, Ninth and Walnut Streets, Philadelphia, PA 19107 (Dr J. Shields).

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