A Case of Weber-Christian Disease with Severe Fatty Liver and Pericardial Effusion / 대한류마티스학회지

ABSTRACT

Weber-Christian disease (WCD) was first described by Pfeifer in 1892, and more clearly defined by Weber and Christian in the 1920s. It is a process of unknown etiology characterized by recurrent fever and inflammation of the adipose tissue. Pathological studies disclose areas of fat necrosis with an inflammatory infiltrate showing a lobular pattern and the usual presence of macrophages with foamy cytoplasm. The clinical signs include tender, palpable nodules, located mainly in the extremities, and fever, abdominal pain, arthritis and arthralgia and hepatosplenomegaly have also been reported. We present a case of Weber-Christian disease in which the presence of multiple subcutaneous nodules, enophthalmos, fatty liver, pericardial effusion was noticed. Biopsy of the skin showed mixed panniculitis in the subcutaneous fat layer. She responded well to glucocorticoid, colchicine and hydroxychloroquine.