A case of Weber?Christian disease associated with myelodysplastic syndrome

Autor: Nobumasa, Hojo, Hitoshi, Hasegawa, Kikue, Iwamasa, Satoko, Hojo, Shigeru, Fujita
Rok vydání: 2004
Předmět:
Zdroj: Modern Rheumatology. 14:73-76
ISSN: 1439-7609
1439-7595
DOI: 10.1007/s10165-003-0270-5
Popis: 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.
Databáze: OpenAIRE
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