Cronkhite-Canada syndrome presenting with chronic diarrhea: A case report

Cronkhite-Canada syndrome, also known as gastrointestinal polyp pigmentation nail dystrophy/atrophy syndrome, is a rare, nongenetic disorder of unknown etiology characterized by gastrointestinal polyps and ectodermal abnormalities. Clinical manifestations commonly include abdominal pain, diarrhea, alopecia, skin hyperpigmentation, nail dystrophy, and dysgeusia. The syndrome may be associated with life-threatening complications, such as gastrointestinal bleeding, intussusception, recurrent pancreatitis, electrolyte disturbances, and hypoproteinemia.

This report presents a 66-year-old man admitted for recurrent abdominal pain and diarrhea. He was initially diagnosed with acute enteritis and treated empirically with antibiotics, which provided only transient relief. His symptoms recurred and progressively worsened >10 days after treatment cessation.

The patient had significant abdominal symptoms with ectodermal changes, and endoscopic and computed tomography computed tomography imaging of the small intestine revealed multiple polyps throughout the gastrointestinal tract (except the esophagus).

The patient was treated with glucocorticosteroids, mesalazine, proton pump inhibitors, rehydration with potassium-containing fluids, and nutritional support.

After treatment with glucocorticoids combined with mesalazine, the abdominal pain and diarrhea improved significantly. However, due to poor compliance, the frequency of diarrhea increased to ten episodes per day after discharge. The patient was readmitted to the hospital due to a recurrence of symptoms and was treated with glucocorticoids and mesalazine. He was discharged from the hospital after his symptoms improved.

In this case, diffuse, variably sized, densely distributed, congestive polypoid changes were seen throughout the stomach, colon, rectum, and the terminal ileum. The lesions were more pronounced in the distal region of the gastric body than in the proximal region. Histopathology was suggestive of hamartomatous polyps with infiltration by eosinophils, lymphocytes, and plasma cells. Hormonal therapy has shown significant efficacy in treating this disease. Timely therapeutic intervention combined with systematic surveillance may mitigate the potential for malignant transformation and disease-related complications.