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A case of Degos disease: demonstration of C5b-9-mediated vascular injury

  • Case Report
  • Published:
Modern Rheumatology

Abstract

A 68-year-old Japanese male presented with atrophic erythematous white lesions with peripheral dark reddish rims on his back. Multiple ulcers were detected from his stomach to his large intestine using endoscopy. Although the patient was given high doses of a steroid, aspirin, dipyridamole, and intravenous immunoglobulin therapy, he died of gastrointestinal hemorrhage, perforation and septic shock. An autopsy examination revealed pauci-inflammatory thrombotic microangiopathy with endothelial cell injury, fibrous occlusive arteriopathy, and vascular C5b-9 deposition in the wall of the gastrointestinal tract from the esophagus to the large intestine as well as in the dermis of the skin.

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Authors and Affiliations

  1. Division of Rheumatology, Department of Medicine, Showa University School of Medicine, 1-5-8 Hatanodai, Shinagawa-ku, Tokyo, Japan

    Masayu Umemura, Yusuke Miwa, Ryo Yanai, Sakiko Isojima, Takahiro Tokunaga, Hiroyuki Tsukamoto, Ryo Takahashi, Nobuyuki Yajima & Tsuyoshi Kasama

  2. Department of Dermatology, Showa University School of Medicine, Tokyo, Japan

    Nanako Takahashi & Hirohiko Sueki

  3. Division of Gastroenterology, Department of Medicine, Showa University School of Medicine, Tokyo, Japan

    Sayaka Yamaguchi, Katsuhito Arai & Yoshiaki Takeuchi

  4. Department of Pathology, Showa University School of Medicine, Tokyo, Japan

    Nobuyuki Ohike, Tomoko Norose, Toshiko Yamochi-Onizuka & Masafumi Takimoto

Authors
  1. Masayu Umemura
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  2. Yusuke Miwa
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  3. Ryo Yanai
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  4. Sakiko Isojima
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  5. Takahiro Tokunaga
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  6. Hiroyuki Tsukamoto
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  7. Ryo Takahashi
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  8. Nobuyuki Yajima
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  9. Tsuyoshi Kasama
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  10. Nanako Takahashi
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  11. Hirohiko Sueki
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  12. Sayaka Yamaguchi
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  13. Katsuhito Arai
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  14. Yoshiaki Takeuchi
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  15. Nobuyuki Ohike
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  16. Tomoko Norose
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  17. Toshiko Yamochi-Onizuka
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  18. Masafumi Takimoto
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Corresponding author

Correspondence to Yusuke Miwa.

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Cite this article

Umemura, M., Miwa, Y., Yanai, R. et al. A case of Degos disease: demonstration of C5b-9-mediated vascular injury. Mod Rheumatol (2013). https://doi.org/10.1007/s10165-013-0888-x

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  • DOI: https://doi.org/10.1007/s10165-013-0888-x

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