姐弟同患色素沉着-息肉综合征

Siblings with pigmentation-polyposis syndrome

报告2例色素沉着-息肉综合征患者。两患者系姐弟,皮损均表现为口唇黑斑,内镜检查均见消化道多发息肉。口唇皮损组织病理可见基底层色素增多,真皮浅层见少量噬色素细胞。家系调查发现该家族3代7人,5人患病,其中男4例,女1例。诊断:色素沉着-息肉综合征。治疗:胃肠多发息肉电凝电切术。

Two patients with pigmented-polyposis syndrome are reported.These two patients are siblings both presented with dark spots on the lips.Endoscopic examination showed multiple polyps in the digestive tract.Histopathological examination of the lip lesions revealed increased pigmentation in the epidermal basal layer,and a small number of pigmentophages were observed in the superficial dermis.Family survey found that among the 7 members(3 generations)in this family,5(4 males and 1 female)were sick.The diagnosis of pigmentation-polyposis syndrome was made and the gastrointestinal multiple polyps were resected by electrocoagulation.