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Orbital Xanthogranulomatous Diseases

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Orbital Inflammatory Diseases and Their Differential Diagnosis

Part of the book series: Essentials in Ophthalmology ((ESSENTIALS))

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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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Compliance with Ethical Standards

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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Authors and Affiliations

  1. Department of Ophthalmology, Eye Plastic and Facial Cosmetic Surgery, Grand Rapids, MI, USA

    Zachary D. Pearce DO & Adam S. Hassan MD

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  1. Zachary D. Pearce DO
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  2. Adam S. Hassan MD
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Correspondence to Zachary D. Pearce DO .

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Editors and Affiliations

  1. Ophthalmology and Visual Sciences, W.K. Kellogg Eye Center, Ann Arbor, Michigan, USA

    Hakan Demirci

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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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  • DOI: https://doi.org/10.1007/978-3-662-46528-8_7

  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-662-46527-1

  • Online ISBN: 978-3-662-46528-8

  • eBook Packages: MedicineMedicine (R0)

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