Cronkhite-Canada syndrome is a rare gastrointestinal disorder characterized by widespread colon polyps, unhealthy looking (dystrophic) nails, hair loss. Cronkhite-Canada syndrome (CCS) is a rare gastrointestinal (GI) polyposis syndrome characterized by the association of non-hereditary GI polyposis with the. Cronkhite-Canada Syndrome (CCS) is a rare non-inherited disease characterized by gastrointestinal polyposis and ectodermal abnormalities.

Author: Zurisar Gardasida
Country: Spain
Language: English (Spanish)
Genre: Career
Published (Last): 22 February 2011
Pages: 168
PDF File Size: 17.92 Mb
ePub File Size: 10.37 Mb
ISBN: 210-1-77897-885-9
Downloads: 81980
Price: Free* [*Free Regsitration Required]
Uploader: Arashijind

Orphanet: Cronkhite Canada syndrome

All studies receiving U. CCS occurs primarily in the older population average age 59 and predominantly dyndrome in males. The Surgical clinics of North America. Together we are strong. Accessed April 2, Azathioprine was initiated with infliximab, at the beginning of therapy to prevent antibody formation to the anti-TNF.

It is characterized by acquired gastrointestinal polyposis with an associated ectodermal triad, including alopecia, onchodystrophy, and hyperpigmentation. One of the most ayndrome mainstays of treatment is aggressive nutritional support with a high protein diet, hyperalimentation, and fluid and electrolyte replacement [ 12 ].

Cronkhite-Canada syndrome

Diagnosis and Management pp. In this report, we describe the presentation and diagnosis of a case of CCS and report encouraging treatment response with anti-TNF therapy. Parikh K and Elston DM. On review of the English literature, there are 59 cases of CCS treated with prednisone with a clear response defined by clinical improvement in symptoms of malabsorption or endoscopic improvement defined primarily by polyp regression.

Cronkhite—Canada syndrome is a rare, sporadic, non-inherited disorder characterised by gastrointestinal and dermatological symptoms.

Antiacid measures including histamine receptor antagonists, proton pump inhibitors, and cromolyn have been used, particularly in patients with biopsies demonstrating eosinophilia [ 13 ]. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.

Other search option s Alphabetical list. Case reports have suggested the use of immunosuppressive treatment, including aziathioprin and ciclosporin, if other treatments are not effective. At 4 months from induction, the patient began to have nail regrowth, improvements in taste, and a modest improvement in diarrhea and weight.


Cronkhite-Canada syndrome — Mayo Clinic

From Wikipedia, the free encyclopedia. Recently, Watanabe et al. Pain management may also be required. Physical examination showed hair regrowth on the scalp, abdomen, and axillary and pubic regions in addition with improved proximal stndrome bed health. Correspondence should be addressed to S.

General Discussion Cronkhite-Canada syndrome CCS is an extremely rare disease characterized by various intestinal polyps, loss of taste, hair loss, and nail growth problems. The inflammatory cell content of the lamina propria was mildly increased with prominent eosinophils. Endoscopic evaluation may reveal multiple sessile polyps in the stomach, small bowel, colon and rectum.

Check this box if you wish to receive a copy of your message. Conkhite management of Cronkhite-Canada syndrome.

Where native intestinal-type surface epithelium remained, it showed a mild increase of intraepithelial lymphocytes and an occasional intraepithelial eosinophil. Springer International Publishing, Cronkhite-Canada syndrome CCS is a rare gastrointestinal GI polyposis syndrome characterized by the association of non-hereditary GI polyposis with the cutaneous triad of alopecia, nail changes and hyperpigmentation.

Genetic counseling CCS occurs sporadically so there is no risk of intrafamilial transmission. A number of studies have reported that timely corticosteroid therapy can facilitate endoscopic regression of the polyposis syndrome resulting in nodular mucosa with a cobblestone appearance, but it is unclear if this translates to a change in the natural history of the disease. Treatment for CCS is largely anecdotal, and randomized controlled therapeutic trials are lacking due to the rarity of the disease.

The gastric polyps were also a characteristic of Cronkhite-Canada syndrome. Other symptoms may include loss of hair alopecialarge areas of dark spots on the skin hyperpigmentation and degenerative changes and, eventually, loss of the fingernails onychodystrophy.

Taylor1 J. These people may also have abnormally low levels of protein in the blood protein-losing enteropathycausing a feeling of general ill health cachexiamalnutrition, nausea and vomiting. Regular colonoscopy to remove polyps and identify colon cancer should be undertaken. It is difficult to treat because of malabsorption that accompanies the polyps.


Diarrhea is usually followed by a variable sequence of onychodystrophy cronhkite onycholysis, thinning of the nail plate, onychoschizia and onychomadesisalopecia initially patchy, rapidly leading to complete hair lossand hyperpigmentation diffuse light-to-dark brownish macules and plaques, and patchy vitiligo distributed on the palms and soles, upper extremities, face, and chest.

Specialised Social Services Eurordis directory. Differential diagnosis Differential diagnosis includes familial adenomatous polyposis, hyperplastic polyposis syndrome, cap polyposis, juvenile polyposis syndrome, Peutz-Jeghers syndrome and Cowden syndrome see these termsas well as lipomatous polyposis, inflammatory polyposis and lymphomatous polyposis.

Management and treatment Current treatments, which can be used individually or in combination, include steroids, nutritional therapy fluid, electrolyte, protein and vitamin supplementation and, if necessary, an canda diet or parenteral nutrition5-aminosalicylate acid, histamine H2 receptor antagonists, anti-tumor necrosis factor alpha agents, immunomodulators, and eradication of Helicobacter pylori.

Alone we are rare. Etiology The pathogenesis of CCS is still elusive but an immune-mediated process has been proposed and this hypothesis is supported by syndroje increased systemic levels of immunoglobulin G4 and antinuclear antibody found in CCS patients as well as the higher frequency of autoimmune disorders associated with CCS.

Cronkhite–Canada syndrome

Early Detection of Cancer. CCS is a rare clinical entity characterized by diffuse gastrointestinal polyposis and unique ectodermal changes of alopecia, hyperpigmentation, and nail dystrophy. Intestinal bleeding and intussusception syndrpme rare but potentially lethal complications of CCS [ 6 ]. Affected Populations Cronkhite-Canada syndrome is an extremely rare disorder that affects males predominantly.