2018
DOI: 10.1177/2324709618765893
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Sirolimus in Refractory Cronkhite-Canada Syndrome and Focus on Standard Treatment

Abstract: Cronkhite-Canada syndrome is a rare syndrome consisting of extensive gastrointestinal polyposis and ectodermal changes including cutaneous hyperpigmentation, alopecia, and onychodystrophy. We report the case of a 45-year-old Caucasian male patient who failed multiple treatments over 2 years including steroids, azathioprine, adalimumab, and cyclosporine. He had recurrent and prolonged hospitalizations because of diarrhea, abdominal pain, weight loss, and malnutrition. Sirolimus was initiated with a significant … Show more

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Cited by 8 publications
(2 citation statements)
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References 15 publications
(16 reference statements)
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“…6,9,10 An autoimmune etiology of CCS has been suggested and case reports have described the beneficial response of immunosuppressive therapies: azathioprine, anti-tumor necrosis factor antibody, cyclosporine, and sirolimus. 1116 This case contributes additional experience to the limited literature on azathioprine as a viable steroid-sparing strategy in CCS.…”
Section: Discussionmentioning
confidence: 84%
“…6,9,10 An autoimmune etiology of CCS has been suggested and case reports have described the beneficial response of immunosuppressive therapies: azathioprine, anti-tumor necrosis factor antibody, cyclosporine, and sirolimus. 1116 This case contributes additional experience to the limited literature on azathioprine as a viable steroid-sparing strategy in CCS.…”
Section: Discussionmentioning
confidence: 84%
“…Fibrofolliculoma is related to FLCN and mTOR signaling ( 101 ). Cronkhite-Canada syndrome can induce alopecia, which may be benefited by the incorporation of sirolimus ( 102 ).…”
Section: Roles Of Mtor Signaling In Hair Diseasementioning
confidence: 99%