Document Detail


A case of Weber-Christian disease associated with myelodysplastic syndrome.
MedLine Citation:
PMID:  17028810     Owner:  NLM     Status:  In-Data-Review    
Abstract/OtherAbstract:
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:
Nobumasa Hojo; Hitoshi Hasegawa; Kikue Iwamasa; Satoko Hojo; Shigeru Fujita
Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Modern rheumatology / the Japan Rheumatism Association     Volume:  14     ISSN:  1439-7595     ISO Abbreviation:  Mod Rheumatol     Publication Date:  2004  
Date Detail:
Created Date:  2006-10-09     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  100959226     Medline TA:  Mod Rheumatol     Country:  Japan    
Other Details:
Languages:  eng     Pagination:  73-6     Citation Subset:  -    
Affiliation:
First Department of Internal Medicine, Ehime University School of Medicine, Shigenobu, Onsen-gun, Ehime, 791-0295, Japan, hitoshih@m.ehime-u.ac.jp.
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