کد مقاله | کد نشریه | سال انتشار | مقاله انگلیسی | نسخه تمام متن |
---|---|---|---|---|
4032931 | 1603035 | 2010 | 6 صفحه PDF | دانلود رایگان |
![عکس صفحه اول مقاله: Orbital Lobular Panniculitis in Weber-Christian Disease: Sustained Response to Anti-TNF Treatment and Review of the Literature Orbital Lobular Panniculitis in Weber-Christian Disease: Sustained Response to Anti-TNF Treatment and Review of the Literature](/preview/png/4032931.png)
Weber-Christian disease is a febrile, relapsing, non-suppurative panniculitis of unknown etiology. Lobular panniculitis is the essential feature in biopsy specimens and evolves through three recognizable stages. We report a case of Weber-Christian disease with bilateral orbital involvement, at different stages, affecting the orbital fat along with enophthalmos in one orbit, and the upper preaponeurotic fat pad in the other. Weber-Christian disease was refractory to treatment with conventional immunosuppressive regimens; however, early inflammatory—but not chronic fibrotic—orbital lesions responded dramatically to anti-tumor necrosis factor (TNF) therapy. A literature review revealed five additional cases of orbital Weber-Christian disease, none treated with anti-TNF antibodies. Of these, four presented initially with proptosis, representing early stages of inflammation, and two subsequently developed enophthalmos, representing late, inactive stage of the disease. Although orbital Weber-Christian disease is rare, ophthalmologists need to be aware of this entity. Depending on the stage of inflammation, Weber-Christian disease should be included in the differential diagnosis of both proptosis and enophthalmos. Anti-TNF antibodies can successfully treat patients at the early inflammatory stage.
Journal: Survey of Ophthalmology - Volume 55, Issue 6, November–December 2010, Pages 584–589