{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,11]],"date-time":"2026-04-11T01:06:21Z","timestamp":1775869581598,"version":"3.50.1"},"reference-count":34,"publisher":"BMJ","issue":"7","content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["BMJ Case Rep"],"accepted":{"date-parts":[[2025,7,6]]},"published-print":{"date-parts":[[2025,7]]},"abstract":"<jats:p>We describe the case of a young adult woman presenting with progressive bilateral proptosis, periorbital soft tissue swelling and eyelid skin lesions. Imaging showed thickened eyelids and enlarged lacrimal glands and extraocular muscles, and a biopsy of the eyelid disclosed a xanthogranulomatous lesion with increased IgG4+ plasma cells. Further investigation did not reveal any associated systemic findings, and the diagnosis of adult-onset xanthogranuloma was made. Periocular lesions and proptosis responded to oral steroids, but symptoms recurred on tapering. Sustained remission was achieved with low-dose corticotherapy. Adult orbital xanthogranulomatous disease comprises a group of rare histiocytic disorders, and pathology is the key to diagnosis. It can be classified into four syndromes with different systemic manifestations and prognoses, adult-onset xanthogranuloma being the least common. Definitive classification is often challenging and requires thorough pathological review and long-term clinical monitoring. Limited evidence is available for the treatment of these conditions, but a conservative approach with immunosuppression is usually considered the best option.<\/jats:p>","DOI":"10.1136\/bcr-2024-264385","type":"journal-article","created":{"date-parts":[[2025,7,22]],"date-time":"2025-07-22T05:10:11Z","timestamp":1753161011000},"page":"e264385","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":1,"title":["Orbital xanthogranulomatous disease: a rare presentation of adult-onset xanthogranuloma"],"prefix":"10.1136","volume":"18","author":[{"given":"Pedro","family":"Sousa Brand\u00e3o","sequence":"first","affiliation":[{"name":"Department of Neuroradiology, Unidade Local de Sa\u00fade de S\u00e3o Jos\u00e9 EPE, Lisboa, Portugal"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ana","family":"Duarte","sequence":"additional","affiliation":[{"name":"Department of Ophthalmology, Unidade Local de Sa\u00fade de S\u00e3o Jos\u00e9 EPE, Lisboa, Portugal"},{"name":"Department of Ophthalmology, Instituto Portugu\u00eas de Oncologia de Lisboa Francisco Gentil EPE, Lisboa, Portugal"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Joaninha Costa","family":"Rosa","sequence":"additional","affiliation":[{"name":"Department of Pathology, Instituto Portugu\u00eas de Oncologia de Lisboa Francisco Gentil EPE, Lisboa, Portugal"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6114-3054","authenticated-orcid":false,"given":"Alexandra","family":"Borges","sequence":"additional","affiliation":[{"name":"Department of Radiology, Instituto Portugu\u00eas de Oncologia de Lisboa Francisco Gentil EPE, Lisboa, Portugal"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"239","published-online":{"date-parts":[[2025,7,21]]},"reference":[{"key":"2025082711461974000_18.7.e264385.1","doi-asserted-by":"publisher","DOI":"10.1136\/bjo.2005.085894"},{"key":"2025082711461974000_18.7.e264385.2","doi-asserted-by":"crossref","first-page":"457","DOI":"10.1016\/j.det.2015.03.010","article-title":"Adult Orbital Xanthogranulomatous Disease: A Review with Emphasis on Etiology, Systemic Associations, Diagnostic Tools, and Treatment","volume":"33","author":"Kerstetter","year":"2015","journal-title":"Dermatol Clin"},{"key":"2025082711461974000_18.7.e264385.3","doi-asserted-by":"crossref","first-page":"31","DOI":"10.1080\/01676830.2019.1590421","article-title":"Adult xanthogranulomatous disease of the orbit: case report of spontaneous regression and review of treatment modalities","volume":"39","author":"Maeng","year":"2020","journal-title":"Orbit"},{"key":"2025082711461974000_18.7.e264385.4","doi-asserted-by":"publisher","DOI":"10.1080\/14397595.2020.1859710"},{"key":"2025082711461974000_18.7.e264385.5","doi-asserted-by":"publisher","DOI":"10.1007\/s10384-014-0352-2"},{"key":"2025082711461974000_18.7.e264385.6","doi-asserted-by":"crossref","first-page":"1","DOI":"10.4103\/JCAS.JCAS_56_17","article-title":"Xanthelasma: An Update on Treatment Modalities","volume":"11","author":"Laftah","year":"2018","journal-title":"J Cutan Aesthet Surg"},{"key":"2025082711461974000_18.7.e264385.7","doi-asserted-by":"crossref","first-page":"400","DOI":"10.1016\/j.ad.2016.12.005","article-title":"Enfermedad xantogranulomatosa orbitaria del adulto. 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