title: A case of Weber-Christian disease associated with myelodysplastic syndrome.
abstract: We report the case of a 73-year-old man with myelodysplastic syndrome (MDS) who developed Weber-Christian disease (WCD). Bone marrow aspirates showed refractory anemia with abnormal karyotypes such as trisomy 8, trisomy 8 and 14, and trisomy 8, 9, and 14. The patient had intermittent fever associated with multiple tender erythematous nodules on the skin. A biopsy sample taken from a nodule revealed focal subcutaneous infiltration of neutrophils and necrotizing fat tissue. We diagnosed the patient as having lobular panniculitis associated with myelodysplastic syndrome. The serum levels of soluble interleukin-2 (IL-2) receptor, interferon-Gamma, IL-1-Beta, IL-6 and tumor necrosis factor-Alpha were elevated in the active state but returned to normal after prednisolone therapy. This finding appears to implicate a T-cell immune response in the pathogenesis of Weber-Christian disease.
authors.affiliation: First Department of Internal Medicine, Ehime University School of Medicine, Shigenobu, Onsen-gun, Ehime, 791-0295, Japan, hitoshih@m.ehime-u.ac.jp.
authors.forename: Nobumasa
authors.initials: N
authors.lastname: Hojo
authors.forename: Hitoshi
authors.initials: H
authors.lastname: Hasegawa
authors.forename: Kikue
authors.initials: K
authors.lastname: Iwamasa
authors.forename: Satoko
authors.initials: S
authors.lastname: Hojo
authors.forename: Shigeru
authors.initials: S
authors.lastname: Fujita