粘膜皮肤色素沉着——胃肠道息肉综合征9例

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粘膜皮肤色素沉着——胃肠道息肉综合征(Peutg-Jeghel氏综合征,简称黑班息肉综合征)病因和发病机理尚未闸明,以特殊分布的黑色素斑点,分布在唇,颊部粘膜,手指及足趾上;胃肠道多发息肉:家族性与遗传性为其3大特点。本文报道9例,其中男5例,女4例。15~60岁,其中2例有母女同患此症。9例患者均有上、下唇及口周色素斑点沉着:口腔颊部占6例,手足指趾,耳垂尚有分布2例。色斑呈黑色或兰褐色,9例中有5例因腹痛来院就诊。钡餐及结肠双对比造影提示有5例为多发性息肉,分布较广泛,除食管,胃以外,小肠、结肠多发,空肠尚有少数息肉3例,9例中有2例并发肠套叠。2例经手术治疗,病理诊断为错构 Mucosal skin pigmentation - The etiology and pathogenesis of gastrointestinal polyposis syndrome (Peutg-Jeghel’s syndrome, abbreviated as Black’s polyposis syndrome) have not yet been established, with a special distribution of melanin spots, distributed in the mucous membranes of the lips and cheeks, Fingers and toes; Multiple polyps of the gastrointestinal tract: familial and hereditary are its three major characteristics. This article reported 9 cases, including 5 males and 4 females. 15 to 60 years old, of which 2 cases of female and female patients have the same disease. All 9 patients had pigmentation on the upper lip, lower lip, and perioral pigmentation: 6 cases were oral cheeks, and fingers and toes were located in the mouth. There were 2 cases of earlobe distribution. The spots were black or blue-brown, and 5 of 9 cases came to the hospital for abdominal pain. Double contrast imaging of barium meal and colon showed that 5 cases had multiple polyps, which was widely distributed. Except for esophagus and stomach, there were multiple small intestine and colon. There were 3 cases of polyps in jejunum and 2 cases of intussusception in 9 cases. 2 cases were treated by surgery, pathological diagnosis was misconstructed
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