Peutz-Jeghers症候群の2例

抄録

We reported two cases of Peutz-Jeghers syndrome whose chief complaints were medical examination of the pigmented spots on the lips, palms and soles.
Case 1: 44 years old man. He had an oldest son (10 years old) and oldest daughter (7 years old) who both had pigmented spots on the lips. At the age of 5 to 6 years, he had noted pigmented spots on the lips. At the age of 42 years, he was admitted to the Kagoshima University Hospital because of an attack of abdominal pain and the diagnosis of Peutz-Jeghers syndrome was made. After he had undergone transverse mesocolone partial excision and polypectomy, he was referred to our clinic.
Case 2: 22 years old woman. Her mother, diagnosed as Peutz-Jeghers syndrome, died of uterus carcinoma at the age of 38 years. At birth, pigmented spots on the lips were noticed and a small intestinal polyp was found at the age of 19 years. After she was diagnosed as Peutz-Jeghers syndrome, she visited our clinic because of the pigmented spots on the lips and a buccal mucosa.
Both cases 1 and 2 had black or dark brown, comparatively clearly bordered spots, 1 to 5mm in diameter, on upper and lower lips, buccal mucosa, palms and soles.
Roentgen examination revealed multiple polyps in colon sigmoideum and the rectum in case 1 and in case 2 a sporadical polyps in the ventriculus, the intestinum tenne and the colon transversum.
The characteristic histopathological findings were epithelial cells proliferation and prominent branching of the lamina muscularis mucosa. In case 2, much melanin pigmentation was mostly observed in the basal cells of the labial mucosa.
The prognosis was not eventful in both cases.