Adult Orbital Xanthogranulomas: Clinical Features and Management

Kıratlı, Hayyam; Kılıç, Meltem; Tarlan, Berçin; Söylemezoğlu, Figen

doi:10.5301/ejo.5000555

Cited by 12 publications

(3 citation statements)

References 18 publications

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“…Clinical appearance of the eyelid lesions in AXG has been variable [ 63 , 64 ]. AXG can also present atypically as an isolated eyelid margin papillary mass similar to our second case [ 65 ].…”

Section: Discussionmentioning

confidence: 99%

Clinicopathological study of ophthalmic cutaneous and mucocutaneous non-langerhans cell histiocytic lesions

Alkatan,

Fatani,

Maktabi

et al. 2024

BMC Ophthalmol

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Background The “C group” of the histiocytic disorders is characterized by non-Langerhans-cell histiocytic lesions in the skin, mucosal surfaces, or both, out of which Juvenile xanthogranuloma (JXG) is the most common typically affecting the skin. The eye is the most common extra-cutaneous site of JXG., we aim at providing our clinical and histopathological experience with this group of diseases including the adult-onset xanthogranuloma (AXG). Methods This is a retrospective cohort study of all patients with the tissue diagnosis of ocular and periocular cutaneous and mucocutaneous non-LCH disorders who presented to us over a period of 25 years (January 1993 to December 2018). Results Twenty patients were diagnosed as “Group C” disease with an age range of 2 months-60.9 years. Eleven patients were females (55%) and nine were males (45%). The involvement was mostly unilateral in 80.9%. All cases fell into the xanthogranuloma family with 11 JXG patients, 8 AXG patients of skin and ocular surface, and one patient with solitary reticulohistiocytoma (SRH). The clinical site of involvement in JXG was primarily in the eyelid in 5 patients (45%), ocular surface lesions in 2 (18%), iris in 2 (18%), choroidal and bilateral orbital lesions in 1 patient each (9%). The group of AXG, presented equally with eyelid lesions in 4/8 and ocular surface lesions in 4/8. The non-Langerhans’ histiocytic infiltrate showed supportive immunohistochemical staining properties (reactive to CD68 marker and negative to S-100 and langerin markers). Conclusion Among the rare histiocytic disorders, xanthogranulomatosis is the commonest and has wide clinical manifestations. Accurate diagnosis needs to be supported by typical histopathological findings. JXG was the commonest in our study with relatively older mean age at presentation and frequent eyelid rather than iris involvement. AXG is often confused with xanthelasma when involving the eyelids with corneal limbal involvement is relatively frequent.

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Section: Discussionmentioning

confidence: 99%

Clinicopathological study of ophthalmic cutaneous and mucocutaneous non-langerhans cell histiocytic lesions

Alkatan,

Fatani,

Maktabi

et al. 2024

BMC Ophthalmol

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“…1 Given the patient's histopathologic findings, history of adult-onset asthma, and lack of other sys- Patients with adult-onset xanthogranulomatous disease frequently present with palpebral swelling, a yellow-orange elevated eyelid, and/or orbital masses. 1,2 Although the yellowish patches are described as xanthelasmalike, true xanthelasmas are rarely associated with xanthogranulomatous disease of the orbit. 3 Although this presentation was suggestive of xanthogranuloma-tous disease, the diagnosis is histopathologic, and a biopsy is required for confirmation.…”

Section: Discussionmentioning

confidence: 99%

“…Patients with adult-onset xanthogranulomatous disease frequently present with palpebral swelling, a yellow-orange elevated eyelid, and/or orbital masses . Although the yellowish patches are described as xanthelasmalike , true xanthelasmas are rarely associated with xanthogranulomatous disease of the orbit .…”

Section: Discussionmentioning

confidence: 99%

Recurrent Bilateral Periorbital Swelling

2018

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A 47-year old woman with a history of adult-onset asthma and waxing and waning bilateral swelling of the upper eyelids presented with greater swelling of the left than right upper eyelids with yellow lesions (Figure 1A). Previous episodes had been treated successfully with oral prednisone, and the patient had numerous recurrences within the past 10 years. According to the patient's report, a biopsy was performed several years ago at an outside institution, and the diagnosis was xanthelasma. She denied visual changes.Visual acuity was 20/40 OU and improved to 20/25 OU with pinhole testing. Intraocular pressures were normal, extraocular motility was full, and the pupils were round and reactive without an afferent pupillary defect. Results of the examination revealed yellow lesions on both eyelids, palpebral swelling greater in the left than the right eye, erythema, ptosis, an S-shaped deformity of the left upper eyelid, and a palpable mass of the left superolateral orbit (Figure 1A). Hertel exophthalmometry showed 2 mm of relative proptosis in the left eye. Results of slitlamp and dilated fundus examinations were unremarkable. A computed tomographic scan showed preseptal soft tissue swelling in both eyes and an extraconal left lacrimal gland mass extending behind the globe (Figure 1B). We contacted the outside institution, and no reports of histopathologic examination of the biopsy specimen were found.

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