J Korean Ophthalmol Soc.  2011 Aug;52(8):975-978. 10.3341/jkos.2011.52.8.975.

Adult Orbital Xanthogranulomatous Disease in a 42-Year-Old Woman

• Chung SJ
• Lew H

• KMID: 2214776
• DOI: http://doi.org/10.3341/jkos.2011.52.8.975

Abstract

PURPOSE
To report a case of adult orbital xanthogranulomatous disease presented as bilateral swelling and yellowish eyelids in a 42-year-old woman who was misdiagnosed with xanthelasma at a dermatologic clinic.
CASE SUMMARY
A 42-year-old woman presented yellowish and swollen eyelids in both eyes of 2.5 year duration. She had no past history of systemic diseases or other ophthalmologic problems. MRI showed heterogeneous eyelid masses and hypertrophic changes of the lacrimal glands in both eyes. There were no abnormalities on chest X-ray exams. The blood cholesterol and low density lipoprotein levels were increased. Incisional biopsy showed many foamy histiocytes, lymphocytes with germinal centers, several touton giant cells, and negative S100/CD 1 staining; all being features consistent with adult onset xanthogranulomatous disease.
CONCLUSIONS
In cases with bilateral swelling and yellowish colored eyelids, adult orbital xanthogranulomatous disease should be evaluated through biopsy and other systemic examinations.

Keyword

Adult orbital xanthogranulomatous disease; Bilateral eyelid swelling; Touton giant cell; Yellowish eyelids

MeSH Terms

Adult
Biopsy
Cholesterol
Eye
Eyelids
Female
Germinal Center
Giant Cells
Histiocytes
Humans
Lacrimal Apparatus
Lipoproteins
Lymphocytes
Orbit
Thorax
Cholesterol
Lipoproteins

Figure

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  Figure 1. Clinical photographs of the patient's eyelid before starting treatment. These pictures show bilateral yellowish eyelid swelling.

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  Figure 2. MRI demonstrated bilateral soft tissue swelling with no bone invasions. (A) T1 image show a 5-mm ovoid hypointense lesion (arrow) at the left upper eyelid, midline. (B) T2 axial image show diffuse swelling with heterogenous signal intensities at bilateral lacrimal glands. (C) T1 axial image show heterogenous soft tissue swelling at bilateral eyelids and preseptal areas.

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  Figure 3. Histologic findings of orbital mass. (A) Variable germinal centers and nodular lymphoid infiltrates in the lacrimal gland (H&E stain, ×100). (B) Touton giant cells (arrow) and many foamy histiocytes in the subcutaneous layer (H&E stain, ×200).

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  Figure 4. Clinical photographs of the patient's eyelids. (A) After excisional biopsy at month 4 and steroid therapy for 1.5 months. (B): After excisional biopsy at month 7 and steroid therapy for 4.5 months. These pictures show decreased yellow colored eyelids and reduction of eyelid swelling.

Reference

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