JKOS Journal of the Korean Ophthalmological Society J Korean Ophthalmol Soc 0378-6471 2092-9374 Korean Ophthalmological Society 10.3341/jkos.2011.52.8.975 jkos-52-975 Original Article Adult Orbital Xanthogranulomatous Disease in a 42-Year-Old Woman ChungSeok Joong MD LewHelen 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 09 2011 07 09 2011 52 8 975 978 18102010 22012011 29042011 Copyright © 2011 Korean Ophthalmological Society 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.

 Adult orbital xanthogranulomatous disease Bilateral eyelid swelling Touton giant cell Yellowish eyelids References Sivak-Callcott JA Rootman J Rasmussen SL Adult xanthogranulomatous disease of the orbit and ocular adnexa: new immunohistochemical findings and clinical review Br J Ophthalmol 2006906028 Guo J Wang J Adult orbital xanthogranulomatous disease: review of the literature Arch Pathol Lab Med 200913319947 Choi HJ Kwon HJ Kim HY A case of posttraumatic necrobiotic xanthogranuloma of the orbit J Korean Ophthalmol Soc 200849 67780 Park SH Rah SH Kim YH Juvenile xanthogranuloma as an isolated corneoscleral limbal mass: a case report Korean J Ophthalmol 200317636 Lee KM Kim NJ Juvenile xanthogranuloma presenting as a nodule of the eyelid J Korean Ophthalmol Soc 20084911736 Frankel RM Capone R Xanthelasmas and xanthomas ― cutaneous clues to systemic lipid disorders Clin Eye Vis Care 1995711728 Hammond MD Niemi EW Ward TP Eiseman AS Adult orbital xanthogranuloma with associated adult-onset asthma Ophthal Plast Reconstr Surg 20042032932 Hayden A Wilson DJ Rosenbaum JT Management of orbital xanthogranuloma with methotrexate Br J Ophthalmol 2007914346 Elner VM Mintz R Demirci H Hassan AS Local corticosteroid treatment of eyelid and orbital xanthogranuloma Trans Am Ophthalmol Soc 20051036973 discussion 734 Figures

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

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.

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).

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.