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-, IL-1-, IL-6 and tumor necrosis factor- 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.