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KMID : 0191120000150020247
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Journal of Korean Medical Science
2000 Volume.15 No. 2 p.247 ~ p.250
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Weber-Christian disease presenting with proptosis: a case report
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Hyun SH
Kang YM/Kim CD/Lee JM/Kim IT/Kim NS
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Abstract
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Weber-Christian disease (WCD) is a rare inflammatory disease of adipose tissue, which is characterized by painful cutaneous nodules and constitutional symptoms. Although any area of the body containing fat can be affected by WCD, the involvement of retrobulbar fat is uncommon and proptosis is a rare presenting manifestation. We report a case who presented with proptosis of the right eye which is accompanied by painful subcutaneous nodules, high fever and myalgia. Biopsies of retrobulbar tissue and suprapubic nodule showed lobular panniculitis with mixed cellular infiltration, mainly composed of histiocytes and lymphocytes. He responded well to high-dose glucocorticoid.
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