Cronkhite-Canada disease: an infrequent diagnosis

A 55-year-old male was admitted with constitutional syndrome and acute diarrhea.

Physical examination revealed alopecia, cutaneous hyperpigmentation and onychodystrophy. Laboratory tests showed iron deficiency. On gastroscopy, diffuse gastric and duodenal involvement was shown, characterized by a congested nodular mucosa with hypertrophy of gastric folds. Ileocolonoscopy demonstrated a mucosa with a cobblestone pattern and two inflammatory polyps. Biopsies pointed out an inflammatory infiltrate and abundant eosinophils in the lamina propria. Additionally, hyperplasia and foveolar dilation in the stomach, villous atrophy in the duodenum and crypt dilation in the colon were found [1] [2] [3].

All these findings were consistent with Cronkhite-Canada disease. Methylprednisolone 1 mg/kg was started with significant improvement. A descending regimen of corticosteroids and Azathioprine 125 mg/day prevented recurrences.

Cronkhite-Canada disease is an acquired and uncommon entity. It typically presents in middle-aged individuals, with gastrointestinal and dermatological manifestations being most frequent. Gastrointestinal symptoms are often nonspecific. Over time, multiple gastrointestinal polyps typically develop, leading to a malabsorptive syndrome.

Its pathophysiology is unknown, although an autoimmune base is suspected. Its prognosis is unfavorable, with a reported 5-year mortality rate of around 50%. Available treatments include systemic corticosteroids, immunosuppressants, budesonide and supportive care. This case has an unusual endoscopic manifestation, likely due to early diagnosis.

Publikationsverlauf

Artikel online veröffentlicht:
27. März 2025

• References

• 1 Cronkhite-Canada syndrome: A case report and literature review
• 2 Liu Y., Zhang L., Yang Y., Peng T.. Cronkhite-Canada syndrome: report of a rare case and review of the literature. The Journal of International Medical Research.. 2020 48 (5). 300060520922427
• 3 Wu Z.-Y., Sang L.-X., Chang B.. Cronkhite-Canada syndrome: from clinical features to treatment. Gastroenterology Report 2020; 8 (5): 333-342