A Case of Neurofibromatosis Type I (von Recklinghausen’s Disease) with Intra-abdominal Bleeding

DOI
  • Munemoto Sawa
    Department of Dermatology, Hyogo Prefectural Amagasaki General Medical Center
  • Fujishima Chieko
    Department of Dermatology, Hyogo Prefectural Amagasaki General Medical Center
  • Hioki Chika
    Department of Dermatology, Hyogo Prefectural Amagasaki General Medical Center
  • Sasaki Hiroka
    Department of Dermatology, Hyogo Prefectural Amagasaki General Medical Center
  • Yoshida Haruka
    Department of Dermatology, Hyogo Prefectural Amagasaki General Medical Center
  • Kudo Hitoshi
    Department of Dermatology, Hyogo Prefectural Amagasaki General Medical Center
  • Kawasaki Yusuke
    Department of Gastrointestinal Surgery, Hyogo Prefectural Amagasaki General Medical Center
  • Shirakata Yoshiharu
    Department of Gastrointestinal Surgery, Hyogo Prefectural Amagasaki General Medical Center
  • Yamamoto Tetsuro
    Department of Pathology, Hyogo Prefectural Amagasaki General Medical Center

Bibliographic Information

Other Title
  • 腹腔内出血をきたした神経線維腫症 1 型の 1 例

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Abstract

<p>Thisreport presentsa 53-year-old woman with neurofibromatosis type 1 (NF-1). She was brought to the emergency room because of sudden onset of abdominal pain. Contrast-enhanced CT on admission showed a superior mesenteric artery aneurysm and intra-abdominal bleeding. Diagnosis of intra-abdominal hemorrhage due to aneurysm rupture was made. During the course of the disease, multiple intra-abdominal hemorrhages occurred, requiring three endovascular treatmentsand two laparotomiesfor partial jejunal resection and pancreaticoduodenectomy. The pathological specimen removed at the time of pancreaticoduodenectomy showed extensive hemorrhage and proliferating neurofibroma cells in the interstitium, including the perivascular area. Major hemorrhage associated with NF-1 vascular lesions is relatively rare, and there have been few reportsfrom the field of dermatology except for those related to diffuse neurofibromas; however,there have been reportsfrom other fields. It is necessary to explain to patients that they should not be unnecessarily anxious, and that they should go to a core hospital where multidisciplinary treatment is available if they require surgery with major invasion. Patients should be transported to a core hospital in case of emergency. Skin Research, 21 : 157-161, 2022 </p>

Journal

  • Hifu no kagaku

    Hifu no kagaku 21 (3), 157-161, 2022

    Meeting of Osaka Dermatological Association/Meeting of Keiji Dermatological Association

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