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

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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-yearold 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.

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Authors and Affiliations

  1. Department of Surgery, Seoul National University Bundang Hospital, 300 Gumi-dong, Bundang-gu, 463-707, Seongnam, Republic of Korea

    Duck-Woo Kim & Sung-Bum Kang

  2. Department of Radiology, Seoul National University Bundang Hospital, 300 Gumi-dong, Bundang-gu, 463-707, Seongnam, Republic of Korea

    Kyung Ho Lee

  3. Department of Pathology, Seoul National University Bundang Hospital, 300 Gumi-dong, Bundang-gu, 463-707, Seongnam, Republic of Korea

    Ghee-Young Choe & So Yeon Park

  4. Republican Scientific Research Center of Emergency Medicine, Tashkent, Uzbekistan

    Mikhaylyuk Nicholay

Authors
  1. Duck-Woo Kim
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  2. Sung-Bum Kang
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  4. Ghee-Young Choe
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Kim, DW., Kang, SB., Lee, K.H. et al. Degos’ disease (malignant atrophic papulosis) as a fatal cause of acute abdomen: Report of a case. Surg Today 38, 866–870 (2008). https://doi.org/10.1007/s00595-007-3728-9

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  • DOI: https://doi.org/10.1007/s00595-007-3728-9

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