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
Lukash WM, Johnson RB, Wentz DK. Gastrointestinal Aspects of Cutaneous and Familial Diseases.. Ann Intern Med. ;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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