Clinical and Endoscopic Response to Anti-Tumor Necrosis Factor-Alpha Antibody Therapy in a Patient With Cronkhite-Canada Syndrome

Abstract

Cronkhite-Canada syndrome (CCS) is an acquired polyposis syndrome with gastrointestinal and extraintestinal manifestations. Given its rarity and lack of standard treatment, diagnosis and treatment are challenging. Steroid therapy and nutritional support are conventional treatments. There is no consensus on management of steroid-refractory cases. Here, we report the diagnosis and treatment course of a 54-year-old Asian male with CCS, whose initial treatment with prednisone 60 mg a day led to partial response and disease flare up during prednisone tapering. The use of infliximab and azathioprine led to promising remission of his symptoms.

Keywords: Cronkhite-Canada syndrome; cutaneous hyperpigmentation; gastroenterology; gastrointestinal polyps; onychodystrophy; steroid-resistant Cronkhite-Canada syndrome.

Figure 1.

Intestinal and extraintestinal clinical manifestation of the patient at presentation: (A) alopecia, hyperpigmentation of skin on the palms and scalp, and onychodystrophy in all digits most notably in thumbs. Multiple polyps accompanying mucosal edema and hyperemia were observed in the (B) stomach and (C) colon.

Figure 2.

Follow-up endoscopic evaluation after treating with infliximab: Significant regression in both polyps’ size and number in the (A) stomach and (B) entire colon.