Abstract
Cronkhite-Canada syndrome (CCS) is a rare, non-inherited polyposis syndrome, characterized by diffuse gastrointestinal (GI) hamartomatous polyposis with unique dermatologic changes including alopecia, skin hyperpigmentation, and nail dystrophy. Patients can typically present with diarrhea, weight loss, protein-losing enteropathy, and nutritional deficiency. However, it can demonstrate diverse other clinical features, usually with poor prognosis. Currently, there are no specific diagnostic criteria established yet. The etiology of CCS is still obscure, but an autoimmune process has been suggested. Here we present an 81-year-old Caucasian female who had clinical presentations, physical exam, imaging, endoscopy and pathology findings that were all consistent with the diagnosis of CCS. We also include a detailed literature review of the other gastrointestinal polyposis syndromes (hamartomatous, adenomatous, hyperplastic and inflammatory polyposis). A high index of suspicion and recognition of the characteristic clinical, endoscopic as well as histopathological findings of CCS, as well as different gastrointestinal polyposis conditions, can help clinicians with more timely and correct diagnosis.
Highlights
The Cronkhite-Canada syndrome (CCS) was a rare, non-hereditary disorder that was originally described inHow to cite this paper: Shen, X.Y., Husson, M. and Lipshutz, W. (2014) Cronkhite-Canada Syndrome: A Case Report and Literature Review of Gastrointestinal Polyposis Syndrome
The syndrome is characterized by diffuse gastrointestinal (GI) hamartomatous polyps, a unique dermatologic triad of alopecia, skin hyperpigmentation, and onychodystrophy, along with clinical features of diarrhea, weight loss, protein-losing enteropathy, and malabsorptive syndrome
European and Asian patients appear to be most frequently affected [3], with 75% of cases being reported in Japan [4]
Summary
The Cronkhite-Canada syndrome (CCS) was a rare, non-hereditary disorder that was originally described in. Her physical examination showed rashes on inner thighs, mild bilateral lower extremity edema, and guaiac positive brown stool Her blood work showed normal hemoglobin concentration with elevated MCV (107 fL), deceased serum potassium (K), total protein and albumin (K 3.1 mmol/L, serum albumin 2.4 g/dL, total protein 4.7 g/dL). Biopsy reported colonic mucosa with glandular dilation, edema, congestion, chronic inflammation, with focal epithelial inflammatory atypia noted (Figure 6). She subsequently had an upper GI with small bowel follow through (SBFT), which revealed diffusely thickened and nodular gastric folds and evidence of innumerable tiny nodules or polyps, in the antrum with mild distal small bowel fold thickening (Figure 7). Computed tomography showed gastric fold thickening, most prominent in distal body and antrum
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