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Degos' disease (malignant atrophic papulosis) as a fatal cause of acute abdomen: report of a case

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Authors
Kim, Duck-Woo; Kang, Sung-Bum; Lee, Kyung Ho; Choe, Ghee-Young; Park, So Yeon; Nicholay, Mikhaylyuk
Issue Date
2008-08-28
Publisher
Springer Verlag
Citation
Surg Today (2008) 38:866–870
Keywords
Degos’ disease; Malignant atrophic papulosis; Small bowel
Abstract
Degos' disease, otherwise known as "malignant atrophic papulosis," is a rare condition characterized by typical cutaneous lesions. Its involvement of the gastrointestinal (GI) tract is usually associated with a poor prognosis. We report a case of Degos' disease with GI involvement, which ultimately caused peritonitis, sepsis, and death, despite all treatment measures. A 59-year old woman was admitted to our hospital with acute generalized abdominal pain. The patient had presented initially with multiple skin lesions 2 years earlier, and even with surgery for small-bowel perforation 10 months before this admission, Degos' disease had not been diagnosed. Explorative laparotomy revealed multifocal, ischemic changes in the small bowel with perforation in the mid-jejunum. After the operation, she suffered recurrent small-bowel fistulas and died within 3 months. In a patient with acute abdominal pain and typical atrophic papules, clinicians should retain a high index of suspicion for Degos' disease with GI involvement, even though it is rare.
ISSN
0941-1291 (print)1436-2813 (online)
Language
English
URI
http://www.springerlink.com/content/102033/
http://hdl.handle.net/10371/3962
DOI
https://doi.org/10.1007/s00595-007-3728-9
https://doi.org/10.1007/s00595-007-3728-9
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College of Medicine/School of Medicine (의과대학/대학원)Pathology (병리학전공)Journal Papers (저널논문_병리학전공)
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