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

Seok Joong Chung; Helen Lew

doi:10.3341/jkos.2011.52.8.975

Journal List > J Korean Ophthalmol Soc > v.52(8) > 1009108

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Chung and Lew: Adult Orbital Xanthogranulomatous Disease in a 42-Year-Old Woman

Original Article

J Korean Ophthalmol Soc 2011;52(8):975-978.

Published online: 7 September 2011

DOI: https://doi.org/10.3341/jkos.2011.52.8.975

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

Seok Joong Chung, MD, Helen Lew, MD

Department of Ophthalmology, Bundang CHA Medical Center, CHA University, Seongnam, Korea

Address reprint requests to Helen Lew, MD Department of Ophthalmology, Bundang CHA Medical Center #351 Yatap-dong, Bundang-gu, Seongnam 463-712, Korea Tel: 82-31-780-5330, Fax: 82-31-780-5333, E-mail: eye@cha.ac.kr

Received 18 October 2010 Revised 22 January 2011 Accepted 29 April 2011

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

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.

Keywords: Adult orbital xanthogranulomatous disease, Bilateral eyelid swelling, Touton giant cell, Yellowish eyelids

References

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Figure 1.

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

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.

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).

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.

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