Weber-Christian disease presenting with intractable fever and periorbital swelling mimicking angioedema

A 22-year-old male patient with Weber-Christian disease (WCD) presenting with periorbital swelling is a very rare occurrence. Lobular panniculitis in the periorbital and anterior tibial skin was diagnosed in this patient, based on the histological findings from biopsy specimens. The differential diagnosis was done before administering systemic corticosteroid. A dramatic response was achieved several days later, but relapse with intractable fever occurred 1 month later. The use of other immunosuppressive agents failed, and he died of septic shock 2 months later. The preceding infection may have been the immunopathologic factor in the development of WCD. Other effective treatments for WCD may be described in the future.