Orbital lobular panniculitis in Weber-Christian disease: sustained response to anti-TNF treatment and review of the literature.
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.
Mavrikakis
Ioannis
I
Department of Ophthalmology, Metropolitan Hospital, Athens, Greece. jmavrikakis@yahoo.com
Georgiadis
Thomas
T
Fragiadaki
Kalliopi
K
Sfikakis
Petros P
PP
D000328:Adult; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use*; D000911:Antibodies, Monoclonal / Q000627:therapeutic use*; D003937:Diagnosis, Differential; D015841:Enophthalmos / Q000175:diagnosis; D005094:Exophthalmos / Q000175:diagnosis; D005260:Female; D006801:Humans; D000069285:Infliximab; D009916:Orbital Diseases / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy*; D010201:Panniculitis, Nodular Nonsuppurative / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy*; D014057:Tomography, X-Ray Computed; D014409:Tumor Necrosis Factor-alpha / Q000276:immunology
D000893:Anti-Inflammatory Agents; D000911:Antibodies, Monoclonal; D014409:Tumor Necrosis Factor-alpha; D000069285:Infliximab