초록 열기/닫기 버튼
Cowden 증후군은 어느 조직에나 발생할 수 있는 과오종을 특징으로 하며 30～50%의 가족력을 가지고 있는 질환이다. 악성 병변의 발생 가능성이 있어 주의를 요하는 질환으로 국내에서는 드물게 보고되고 있다. 임상 증상은 병변의 위치에 따라 다르지만 소화기 용종증의 증상으로는 출혈, 설사, 복통 등이 있다. 저자 등은 최근 빈혈을 주소로 내원하여 구각 각화증, 갑상선 양성 결절, 식도의 극세포증 및 소화관 용종증 등의 증상으로 Cowden 증후군을 진단한 1예를 경험하였기에 문헌 고찰과 함께 보고하는 바이다.
A 17-year-old man was referred to our hospital because of dizziness and mild dyspnea. He had a history of partial thyroidectomy due to benign mass when he was 10 year-old. His conjunctiva was anemic and acral keratosis was noted. He had a goiter and a previous surgical scar on the anterior neck just below the thyroid cartilage. Blood counts were as follows; Hb was 4.3 g/dL, WBC 5,500/mm3, PLT 366,000/ mm3. Esophagogastroduodenoscopy and colonoscopy showed esophageal acanthosis and numerous variable sized polyps in the stomach, duodenum and colon. The small bowel series showed numerous polypoid lesions in the entire small bowel. Gastric polyps revealed hamartoma and colon polyps revealed inflammatory polyp with lymphoid hyperplasia by pathologic examination. Thyroid ultrasonograph showed multiple nodules and cysts and I131 scintigraphy showed multiple cold and hot nodules in his remnant thyroid gland. We diagnosed the patient as Cowden's syndrome. (Korean J Gastrointest Endosc 2002;24:17-20)
키워드열기/닫기 버튼
Cowden's syndrome, Acral keratosis, Thyroid nodules, Gatrointestinal polyposis
