Comprehensive treatment and a rare presentation of Cronkhite–Canada syndrome: Two case reports and review of literature

BACKGROUND Cronkhite–Canada syndrome(CCS)is a rare sporadic polyposis syndrome that presents with gastrointestinal and ectodermal symptoms in addition to nutritional deficiencies.CCS combined with hypothyroidism is an even rarer condition,with no standard treatment guidelines.CASE SUMMARY The present study described 2 patients with CCS:A 67-year-old woman with concomitant hypothyroidism and 68-year-old man treated with endoscopic mucosal resection(EMR).Both patients had multiple gastrointestinal symptoms and ectodermal changes,along with multiple gastrointestinal polyps.Microscopic examination showed that the mucosa in both patients was hyperemic and edematous,with pathologic examination showing distorted,atrophic,and dilated glands.Patient 1 had concomitant hypothyroidism and was treated with levothyroxine.Due to her self-reduction of hormone dose,her disease relapsed.Patient 2 underwent EMR,but refused further hormonal or biological treatments.Subsequently,he was treated with an oral Chinese medical preparation.CONCLUSION Pharmacotherapy can induce and maintain remission in CCS patients,with adjuvant EMR,long-term follow-up,and endoscopic surveillance being necessary.Case 1:Based on the aforementioned findings,Patient 1 was diagnosed with CCS and hypothyroidism.Case 2:Based on the aforementioned findings,Patient 2 was diagnosed with CCS.

Cronkhite-Canada综合征:临床特征和治疗

Cronkhite-Canada综合征(CCS)是一种罕见的病因不明的获得性息肉病。目前全球已有超过500例的报道。CCS的典型表现是胃肠道症状(如腹泻)和皮肤改变(如脱发、色素沉着和指甲萎缩)。内镜特征包括分布于除食管外整个胃肠道的弥漫性息肉,这些息肉的病理类型主要包括炎性、增生性、错构性及腺瘤性息肉。CCS可并发许多疾病,而且有一定的恶性倾向,病死率较高。目前CCS尚无统一的标准治疗方案。本文对目前所有报道的CCS病例进行综述,旨在提高对该病的认识。