皮肤粘膜色素沉着-胃肠道多发性息肉综合征(PJS)是一种少见病。国内见诸报告者不过效十例。现将我院收治的2例报告如下。病历摘要例1,女,7岁。自2岁开始口腔粘膜及口周皮肤出现许多大小不等的褐色斑,渐扩展至手指尖及足趾尖。1年前开始经常腹痛、发烧,并伴贫血,曾多次住当地医院均未明确诊断。4天前腹痛又发作,呈持续性,伴恶心,呕吐,吐物为胃内容。大便呈暗红 Mucosal pigmentation of the skin - Gastrointestinal polyposis syndrome (PJS) is a rare disease. However, there are only 10 cases reported by domestic reporters. Now in our hospital admitted 2 cases are as follows. Case history summary 1, female, 7 years old. Since the age of 2 oral mucosa and perioral skin appeared many sizes of brown spots, gradually extended to the tip of your fingers and toe tip. A year ago began abdominal pain, fever, and anemia, have repeatedly lived in a local hospital were not a clear diagnosis. 4 days ago abdominal pain and attack, was persistent, with nausea, vomiting, spit things for the stomach. Dark red stool