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Xanthogranulomatous Disease of the Orbit: Case Report

Miljenka Tojagić
Irena Vladušić
Mirjana Čačić
Neda Stiglmayer

Abstract

Xanthogranulomatosis is a rare idiopathic histiocytic disorder, histologically characterized by xanthogranulomatous inflammation. It mostly affects bones, heart, lung, liver, kidneys, retroperitoneum, brain, and rarely the orbit. In the literature, it is mentioned as Erdheim-Chester disease. Ophthalmic involvement has been reported in less than 30 cases. We describe a 46-year-old man with unilateral exophthalmos and xanthogranulomatous inflammation of periorbital area, temporal area and face bilaterally, associated with asthma and atopic dermatitis. Dexamethasone was administered retrobulbarly. This therapy resulted in a significant reduction of exophthalmos and edema of the surrounding tissue, which improved the patient's quality of life. There was no recurrence of inflammation during the one-year follow up. It is concluded that treatment with steroids provides good results in the management of xanthogranulomatous inflammation of the orbit.

Keywords

Xanthogranulomatosis; Orbitopathy; Steroids

Hrčak ID:

22813

URI

https://hrcak.srce.hr/22813

Publication date:

1.4.2008.

Article data in other languages: croatian

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