Orbital adult-onset xanthogranuloma is a rare idiopathic inflammatory disorder included in the group of orbital xanthogranulomatous diseases with possible systemic associations. A 43 year-old woman presented with a deep fast-growing mass on the right upper eyelid. She had a past medical history of bilateral non-ulcerative nodules on the upper eyelid and two cosmetic upper lid blepharoplasties. An excisional biopsy was performed and the histopathology analysis confirmed the diagnosis of adult histiocytic xanthogranuloma. The systemic work up was negative and the patient is asymptomatic without treatment. Orbital xanthogranulomatous diseases are rare, but potentially life-threatening disorders. The ophthalmologist must be aware of this disease in order to make an early diagnosis using the anamnesis, clinical examination, and histology of the periocular lesions that may resemble palpebral xanthelasmas.
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http://dx.doi.org/10.1016/j.oftal.2018.12.004 | DOI Listing |
J Ophthalmic Vis Res
June 2024
Ophthalmic Research Center, Research Institute for Ophthalmology and Vision Science, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
Purpose: To report a case of adult-onset asthma and periocular xanthogranuloma (AAPOX) in a patient with intermediate uveitis and a history of Hodgkin's lymphoma (HL).
Case Report: A 51-year-old man with a past medical history of HL presented with blurred vision, asthma, and bilateral yellowish eyelid lesions. The eyelid lesions and asthma appeared 10 years after being diagnosed with HL.
Int Ophthalmol
July 2024
School of Medicine, University of Zagreb, Šalata 3B, Zagreb, Croatia.
Purpose: Within the large umbrella of histiocytosis are a few similar yet heterogenous entities involving the orbit and periocular tissues with or without systemic infiltration, termed adult onset xanthogranuloma or orbital xanthogranuloma. Due to rarity of these conditions, different classifications in use, diverse clinical presentations and still unknown etiology, the aim of this paper was to provide an up-to-date literature review of the actual understanding of histiocytosis and its subgroups involving the orbit and periocular area, diagnostic strategies and therapeutic modalities.
Methods: We present a review of literature and small case series comprising four patients diagnosed and treated in the period from 2001 until 2023 in our hospital.
Clin Immunol
August 2024
The Rotterdam Eye Hospital, Rotterdam, the Netherlands; Department of Pathology, Leiden University Medical Center, Leiden, the Netherlands; Department of Pathology, section Ophthalmic Pathology, Erasmus MC University Medical Center Rotterdam, Rotterdam, the Netherlands. Electronic address:
Adult orbital xanthogranulomatous disease (AOXGD) is a spectrum of histiocytoses with four subtypes. Mitogen-activated protein kinase (MAPK) pathway mutations have been detected in various histiocytic neoplasms, little is known about this in AOXGD. Targeted regions of cancer- and histiocytosis-related genes were analyzed and immunohistochemical staining of phosphorylated ERK (pERK), cyclin D1 and PU.
View Article and Find Full Text PDFAm J Ophthalmol Case Rep
September 2024
Department of Ophthalmology, CHA Bundang Medical Center, CHA University, Seongnam-si, Republic of Korea.
Purpose: We report a patient who initially visited the ophthalmology clinic for a vision loss diagnosed with Erdheim-Chester Disease (ECD).
Observations: ECD is a rare non-Langerhans cell histiocytosis characterized by multisystemic organ involvement and poor prognosis. Our patient had complete vision loss due to prominent orbital involvement before any systemic symptoms appeared.
Ann Allergy Asthma Immunol
July 2024
Division of Allergy, Immunology and Rheumatology, Department of Medicine, Rutgers New Jersey Medical School, Newark, New Jersey.
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