William M. Lukash, M.D., F.A.C.P.; Raymond B. Johnson, M.D.; Dennis K. Wentz, M.D.
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Unusual gastrointestinal manifestations may develop in certain heritable disorders commonly associated with distinctive cutaneous features. The following group of familial diseases, with illustrative case histories, are described: von Recklinghausen's disease, Ehlers-Danlos syndrome, hereditary hemorrhagic telangiectasia, Peutz-Jeghers syndrome, and primary amyloidosis. In most of these conditions gastrointestinal bleeding was common, and lesions were seen and photographed during fiber-optic gastroscopy. A variety of gastrointestinal tumors was seen in von Recklinghausen's disease and included neurofibroma, leiomyoma, neurofibrosarcoma, and adenomatous polyps. Gastrointestinal polyps in Peutz-Jeghers syndrome were associated with protein-losing enteropathy and a mixed anemia. Intestinal obstruction due to intraluminal tumor encroachment and intussusception
William M. Lukash, Raymond B. Johnson, Dennis K. Wentz. Gastrointestinal Aspects of Cutaneous and Familial Diseases.. Ann Intern Med. 1969;70:1069–1070. doi: 10.7326/0003-4819-70-5-1069_3
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Published: Ann Intern Med. 1969;70(5):1069-1070.
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Print ISSN: 0003-4819 | Online ISSN: 1539-3704
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