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

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,...

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Bibliographic Details
Published in:Modern rheumatology 2015-05, Vol.25 (3), p.480-483
Main Authors: Umemura, Masayu, Miwa, Yusuke, Yanai, Ryo, Isojima, Sakiko, Tokunaga, Takahiro, Tsukamoto, Hiroyuki, Takahashi, Ryo, Yajima, Nobuyuki, Kasama, Tsuyoshi, Takahashi, Nanako, Sueki, Hirohiko, Yamaguchi, Sayaka, Arai, Katsuhito, Takeuchi, Yoshiaki, Ohike, Nobuyuki, Norose, Tomoko, Yamochi-Onizuka, Toshiko, Takimoto, Masafumi
Format: Article
Language:English
Subjects:
Aged
Autopsies
Complement Membrane Attack Complex - metabolism
Drug therapy
Fatal Outcome
Gastroenterology
Gastrointestinal Tract - metabolism
Gastrointestinal Tract - pathology
Humans
Immunoglobulins
Male
Malignant Atrophic Papulosis - diagnosis
Malignant Atrophic Papulosis - metabolism
Malignant Atrophic Papulosis - pathology
Skin - metabolism
Skin - pathology
Thrombosis
Ulcers
Vein & artery diseases
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Summary: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.
ISSN:1439-7595
1439-7609
DOI:10.3109/14397595.2013.874761