Successful Treatment of a Patient with Febrile, Lobular Panniculitis (Weber-Christian Disease) with Oral Cyclosporin A. Implications for Pathogenesis and Therapy.
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- IWASAKI Tsuyoshi
- The Second Department of Internal Medicine, Hyogo College of Medicine
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- HAMANO Teruaki
- The Second Department of Internal Medicine, Hyogo College of Medicine
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- OGATA Atsushi
- The Second Department of Internal Medicine, Hyogo College of Medicine
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- HASHIMOTO Naoaki
- The Second Department of Internal Medicine, Hyogo College of Medicine
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- KAKISHITA Eizo
- The Second Department of Internal Medicine, Hyogo College of Medicine
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We report a 15-year-old Japanese girl with severe systemic Weber-Christian disease (WCD) who presented with acute onset of high fever associated with tender subcutaneous nodules. Laboratory tests showed an elevated serum concentration of lactate dehydrogenase (LDH), leukopenia, and coagulation abnormalities. The anti-nuclear and anti-DNA antibodies were negative, and the serum pancreatic enzymes and alpha 1-antitrypsin levels were normal. Pulse steroid therapy was not effective, and eventually cerebellar hemorrhage occurred. After initiation of oral cyclosporin A (CyA) therapy, fever came down and her clinical condition improved markedly. Extremely high serum concentrations of interferon-gamma (IFN-γ) and soluble interleukin-2 receptor (sIL-2R) in this patient returned to normal with CyA therapy. These findings suggest that T-cell immune responses are involved in the pathogenesis of WCD, and that CyA is effective against the disease via suppression of T-cell reactions.<br>(Internal Medicine 38: 612-614, 1999)
収録刊行物
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- Internal Medicine
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Internal Medicine 38 (7), 612-614, 1999
一般社団法人 日本内科学会
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詳細情報
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- CRID
- 1390282679846104064
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- NII論文ID
- 10005529641
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- NII書誌ID
- AA10827774
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- COI
- 1:STN:280:DyaK1MzmtFOhtg%3D%3D
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- ISSN
- 13497235
- 09182918
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- NDL書誌ID
- 4792054
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- PubMed
- 10435371
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- 本文言語コード
- en
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- データソース種別
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- JaLC
- NDL
- Crossref
- PubMed
- CiNii Articles
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- 抄録ライセンスフラグ
- 使用不可