|
A Rare Cause Of Fever Of Unknown Origin: Weber-christian Disease
Mesut Başak*, Sabahattin Gül**, Alp Günay***,Zeki ÇankIr****, Şükrü YILDIRIM*****, Mehmet DanacI**
* Yrd.Doç.Dr.,GATA Haydarpaşa Eğitim Hastanesi, Genel Dahiliye Kliniği,** Doç.Dr.,GATA Haydarpaşa Eğitim Hastanesi, Genel Dahiliye Kliniği,*** Uz.Öğr.Dr.,GATA Haydarpaşa Eğitim Hastanesi, İç Hastalıkları Kliniği,**** Uz.Dr.,GATA Haydarpaşa Eğitim Hastanesi, İç Hastalıkları Kliniği,***** Uz.Dr.,GATA Haydarpaşa Eğitim Hastanesi, Patoloji Kliniği, İSTANBUL
Weber-Christian Disease is a systemic inflammation of subcutaneous fat. Prominent clinical features included lower extremity subcutaneous nodules, fever, arthritis/arthralgia and myalgia. It is frequently encountered in females and middle-aged people. Notable laboratory features are elevated erythrocyte sedimentation rate, anemia, leukopenia and hypocomplementemia. Histologic findings are lobular panniculitis and "fat-laden" macrophages. Classically described Weber-Christian Disease is a syndromes of diverse etiology that share many clinical, inflammatory, and immunologic features. The most effective agents in the treatment are anti-malarials, corticosteroids and immunosuppressives.
We presented a case of Weber-Christian Disease in which the predominant symptoms were fever and bleeding, there were no response to classic treatment, remission was maintained by desmopressin acetate and tranexamic acid therapy, and relevant literature has been reviewed.
Keywords: Weber-Christian disease, Fever of unknown
origin, Bleeding, Treatment
Turkiye Klinikleri J Med Sci 1998, 18:322-327
|
