Fischer R, Breidert M, Paul S, Kreisel W, Schmitt-Gräff A, Waller C, Blum H E
Medizinische Abteilung II, Universitätsklinikum Albert-Ludwigs-Universität Freiburg i. Br.
Dtsch Med Wochenschr. 2010 Feb;135(8):339-42. doi: 10.1055/s-0029-1244856. Epub 2010 Feb 17.
A 70-year-old man with a 14 month history of chronic severe diarrhea treated with budenosid and mesalazin was admitted because of peripheral oedema and weight loss of about 26 kg.
A general pigmentation of the skin, especially on scull and hands, as well as dystrophic nail changes and alopecia of scalp and facial hair were seen. The tests showed a slight macrocytotic, normochromic anemia. Total protein and the clotting factors were decreased. Endoscopy revealed multiple sessile polyps in the stomach and duodenum in appearance to colon and rectum. Endoscopic removal of a polyp showed histologically cystic dilatation of foveolae and oedematous mucosa. The histological features, the wide distribution of the polyps together with the skin changes, lead to the diagnosis of Cronkhite-Canada syndrome (CCS).
The patient was initially treated with prednisolon 60 mg/d i. v. for 2 weeks, resulting in a marked improvement of symptoms and weight gain. He is at present in good health under prednisolon 20 mg/d per os and is followed up in our outpatient department.
CCS is in up to 14 % of the cases associated with a carcinoma of the gastrointestinal tract. At present there are only reports about a successful treatment by steroids, prophylactic gastrectomy and proctocolectomy. Typical myopathic lesions of CCS have not been described to date, but the demonstrated improvement of creatinin kinase with successful treatment suggests a common pathophysiological mechanism.
