Abstract
Xanthogranulomatous diseases of the eyelids and orbit represent a rare group of histiocytic, granulomatous disorders similar in their predominant cell types but diverse in their prognosis – from benign and self-limited to systemically malignant and ultimately fatal. Distinct clinical syndromes involving xanthogranulomatous inflammation have been described, but clinical overlap exists, suggesting that they are part of a continuum. As the name implies, juvenile xanthogranuloma (JXG) affects children. In adults, the four syndromes of xanthogranulomatous inflammation in the eye and adnexa are adult orbital xanthogranuloma (AOX), adult-onset asthma and periocular xanthogranuloma (AAPOX), necrobiotic xanthogranuloma (NXG), and Erdheim-Chester disease (ECD). Each of these entities is reviewed in detail herein.
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Zachary D. Pearce and Adam S. Hassan declare that they have no conflict of interest. No human studies were carried out by the authors for this article. No animal studies were carried out by the authors for this article.
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Pearce, Z.D., Hassan, A.S. (2015). Orbital Xanthogranulomatous Diseases. In: Demirci, H. (eds) Orbital Inflammatory Diseases and Their Differential Diagnosis. Essentials in Ophthalmology. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-46528-8_7
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