J Korean Ophthalmol Soc.  2002 Jul;43(7):1323-1329.

Erdheim-Chester Disease: Two Cases of Orbital Involvement

• Kim YJ
• Kim YD

Abstract

PURPOSE: ECD is a rare idiopathic condition characterized by infiltration of the bone, heart, lungs, retroperitoneum, skin, CNS and other tissues by a fibrosing process containing xanthomatous histiocytes and multinucleated giant cells of the Touton type. Orbital involvement of ECD is rare and we report two cases of Erdheim-Chester disease.
METHODS
A 46-year-old man with dilated cardiomyopathy and chronic renal failure and a 70-year-old woman with pulmonary disease and intracranial mass visited our clinic because of bilateral proptosis.
RESULTS
The systemic evaluation revealed multiple organ involvement, such as bone, heart, lung and retroperitoneum ,while radiography of the tibia showed symmetrical osteosclerosis. The biopsy of tibia revealed a diffuse infiltration of foamy histiocytes and multinucleated giant cells of the Touton type. Based on the diagnosis of ECD, the patients were treated with radiation and steroids.
CONCLUSIONS
Although most patients with ECD do not have ocular involvement, the unusual occurrence of bilateral proptosis and diffuse infiltrative orbital masses are suggestive of ECD. These findings should alert the ophthalmologist to this potentially serious systemic disease and prompt an evaluation for systemic manifestations.

Keyword

Erdheim-Chester disease; Orbital mass; Proptosis

MeSH Terms

Aged
Biopsy
Cardiomyopathy, Dilated
Diagnosis
Erdheim-Chester Disease*
Exophthalmos
Female
Giant Cells
Heart
Histiocytes
Humans
Kidney Failure, Chronic
Lung
Lung Diseases
Middle Aged
Orbit*
Osteosclerosis
Radiography
Skin
Steroids
Tibia
Steroids