Mucocutaneous Manifestations of Gastrointestinal Polyposis Syndromes Atsushi Hirano 1 , Junji Umeno 1 , Yuji Maehata 1 , Takehiro Torisu 1 , Tomohiko Moriyama 1 , Takayuki Matsumoto 2 , Motohiro Esaki 1 1Department of Medicine and Clinical Science, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan 2Division of Gastroenterology, Department of Internal Medicine, School of Medicine, Iwate Medical University, Iwate, Japan Keyword: 家族性大腸腺腫症 , Peutz-Jeghers症候群 , Cowden症候群 , Cronkhite-Canada症候群 , 若年性ポリポーシス/遺伝性出血性末梢血管拡張症複合症候群 , 皮膚病変 pp.999-1008
Published Date 2016/7/25
DOI https://doi.org/10.11477/mf.1403200682
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 Here the clinical features of GI(gastrointestinal)polyposis syndromes that can manifest as mucocutaneous lesions have been described. Among GI polyposis syndromes, FAP(familial adenomatous polyposis), PJS(Peutz-Jeghers syndrome), CS(Cowden syndrome), CCS(Cronkhite-Canada syndrome), and JPs-HHT(juvenile polyposis-hereditary hemorrhagic telangiectasia)overlap syndrome are frequently accompanied by mucocutaneous lesions. Epidermoid cysts and lipoma in FAP ; mucocutaneous pigmentations in PJS ; facial papules, gingival papillomas, and acral keratosis in CS ; alopecia, onychodystrophy, and cutaneous hyperpigmentation in CCS ; and mucocutaneous telangiectasia in JPs-HHT overlap syndrome are the major mucocutaneous lesions. Being familiar with the mucocutaneous lesions appears to be important for diagnosing GI polyposis syndromes.

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