Sestre Milosrdnice University hospital and Institute of Clinical Medical Research
Abstract
Ksantogranulomatoza je rijetka idiopatska histiocitična bolest histološki obilježena ksantogranulomatoznom upalom. Najčešće zahvaća kosti, srce, pluća, jetru, bubrege, retroperitoneum, mozak, te rijetko orbitu. U literaturi se spominje kao Erdheim-Chesterova bolest. Zahvaćenost očiju opisuje se u manje od 30 slučajeva. Mi prikazujemo 46-godišnjeg muškarca s jednostranim egzoftalmusom i ksantogranulomatoznom upalom periorbitalnog područja, temporalnog područja i lica obostrano, udružene s astmom i atopičnim dermatitisom. U terapiji je primijenjen deksametazon retrobulbarno. Ova terapija je dovela do značajnog smanjenja egzoftalmusa i edema okolnog tkiva, čime je poboljšana bolesnikova kvaliteta života. Upala se nije ponavljala tijekom jednogodišnjeg praćenja. Zaključuje se kako je terapija steroidima uspješna u liječenju ksantogranulomatozne upale orbite.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\u27s 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
