[The Cronkhite-Canada Syndrome. A rare differential diagnosis of generalized gastrointestinal polyposis]

Abstract

History and findings: A 56-year-old man was admitted to hospital because of chronic diarrhoea. 15 months earlier he had begun to notice changes in taste sensation, then nail discoloration and dystrophy as well as alopecia areata. On examination he was also found to have lower leg oedema and cutaneous hyperpigmentation.

Investigations: Biochemical tests showed hypoproteinaemia with reduced serum total protein (4.2 g/dl) and albumin concentrations (2.0 g/dl), hypokalemia and hypocalcaemia, as well as zinc and vitamin B12 deficiency. Stool alpha 1-antitrypsin was raised to 5.9 mg/g. Erythrocyte sedimentation rate was 17/26 mm and C-reactive protein was raised to 6.9 mg/dl. Gastroscopy, coloscopy and small-intestine double contrast radiology (after Sellink) demonstrated multiple polyps, histologically revealing pseudopolypoid-inflammatory changes with cystic dilatation, consistent with Cronkhite-Canada syndrome (CCS), a condition characterised by the described endoscopic, radiological and histomorphological changes together with the characteristic ectodermal abnormalities.

Treatment and course: At first only symptomatic measures were taken: fluid, electrolyte and protein infusions and administration of zinc and vitamin B12. Stool frequency was regulated by diet and medication. The patient was discharged in much improved general condition and closely followed clinically and endoscopically because of the relatively poor prognosis and frequent occurrence of adenomatous polyps which are at a high risk of malignant degeneration.

Conclusions: In the differential diagnosis of generalised intestinal polyposis only careful investigation by endoscopy and radiology of the entire gastrointestinal tract with biopsies can identify CCS. While treatment is largely symptomatic, its poor prognosis calls for new therapeutic measures.