S4986 Cronkhite-Canada Syndrome: An Inflammatory Bowel Disease Mimic
Bibliographic record
Abstract
Introduction: Cronkhite-Canada syndrome (CCS) is an extremely rare, non-hereditary polyposis syndrome, characterized by diffuse gastrointestinal hamartomatous polyps, along with ectodermal abnormalities such as alopecia, onychodystrophy, and skin hyperpigmentation. There are fewer than 500 cases reported worldwide since it was first described in 1955. We present a case of CCS initially diagnosed as Crohn’s disease highlighting its unique clinical and endoscopic features. Case Description/Methods: A 60-year-old African American woman presented with a 2-year history of watery diarrhea, abdominal pain, as well as progressive weight loss and malnutrition requiring enteral feeding. She was diagnosed as having Sjogren’s syndrome as well as Crohn’s disease and was treated with adalimumab and azathioprine following evaluation by multiple specialists but failed to improve and subsequently sought a second opinion at our institution. Her symptoms were also associated with alopecia, nail loosening, and hyperpigmentation. Laboratory markers and stool studies were unremarkable except for marked eosinophilia. Esophagogastroduodenoscopy revealed a normal esophagus, and markedly edematous, erythematous, and nodular gastric mucosa, most prominent in the body and antrum, along with diffuse nodular mucosa throughout the duodenum. Sigmoidoscopy showed similar findings in the rectum and sigmoid colon. Gastric, duodenal, and colonic biopsies confirmed the presence of hamartomatous polyps. Discussion: CCS is most commonly reported in middle-aged adults. Its etiology remains unclear, though an autoimmune component has been suggested given its association with other autoimmune conditions and increased levels of IgG-4 positive plasma cells in polyps. Recent transcriptomic analyses of CCS polyps have also shown upregulation of innate immune pathways. Early recognition is critical, as delayed diagnosis can lead to significant nutritional deficiencies and increased gastrointestinal malignancy risk and morbidity. Treatment entails nutritional support as well as corticosteroids, with gradual tapering based on clinical response, and steroid-spearing agents like azathioprine. Most patients experience improvement in both gastrointestinal symptoms and ectodermal abnormalities with treatment. Our case underscores the importance of considering CCS in patients presenting with gastrointestinal symptoms suspicious for Crohn’s disease. The presence of typical dermatologic features can lead to an earlier diagnosis.
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How this classification was reachedexpand
Full frame machine prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. The Gemma side is a direct model label for every work in the frame, read from the title-only record. The Codex side is a classifier learned from the 10,348 direct Codex labels and calibrated to design-weighted sample rates; fields without enough sample support carry no Codex call. Candidate is the union of the two sides; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels.
Distilled classifier scores by category (both heads)
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.000 | 0.001 |
| Meta-epidemiology (narrow) | 0.001 | 0.000 |
| Meta-epidemiology (broad) | 0.001 | 0.000 |
| Bibliometrics | 0.002 | 0.002 |
| Science and technology studies | 0.001 | 0.001 |
| Scholarly communication | 0.001 | 0.001 |
| Open science | 0.001 | 0.001 |
| Research integrity | 0.002 | 0.001 |
| Insufficient payload (model declined to judge) | 0.004 | 0.001 |
Machine scores (provisional)
The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.
Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.
score_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from itClassification
machine, unvalidatedMachine predicted; a candidate call from one source (direct Gemma or distilled Codex), not a consensus.
How this classification was reached, model by model and score by score, is at the end of the page under "How this classification was reached".