患者女性,31岁。上腹痛伴恶心,呕吐5天,加重1天入院。查体:口唇周围,颊粘膜、牙龈、手指、手掌、足底、肛周等处有色素沉着,为棕黄色斑块。腹平坦,上腹部扪及一约10 cm×6 cm大小蜡肠样包块。CT检查示上腹部有一双圆形软组织块影,中心有气体,气体外呈同心圆样低密度改变。行空气灌肠气体顺利 Female patient, 31 years old. Upper abdominal pain was associated with nausea, vomiting for 5 days, and hospitalization for 1 day. Physical examination: pigmentation is present around the lips, buccal mucosa, gums, fingers, palms, soles, perianal area, etc., as brown patches. The abdomen is flat, with a waxy bowel mass about 10 cm x 6 cm in size on the abdomen. CT examination showed that there was a pair of circular soft-tissue shadows on the upper abdomen, with gas in the center and concentric low-density changes outside the gas. Air enema gas goes smoothly