Cronkhite-Canada Syndrome

Comments:
Cronkhite-Canada Syndrome (CCS): This is a rare, sporadic polyposis syndrome with no known genetic defects. The underlying pathogenesis is related to immune dysregulation. Patients are seriously ill and present with malabsorption, diarrhea, protein-losing enteropathy and dermal manifestations (alopecia, onychodystrophy, and hyperpigmentation). Treatment usually consists of corticosteroids or cyclosporine (for steroid-resistant cases). Mortality is as high as 50%.

Endoscopy reveals hamartomatous, inflammatory-type polyps throughout the gastrointestinal tract except for esophagus. This endoscopic image shows diffuse involvement of colon.

Image courtesy of: @PatholWalker