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: Akinorn Akir
Country: Andorra
Language: English (Spanish)
Genre: Medical
Published (Last): 25 September 2006
Pages: 39
PDF File Size: 15.19 Mb
ePub File Size: 12.4 Mb
ISBN: 213-4-69971-560-1
Downloads: 97016
Price: Free* [*Free Regsitration Required]
Uploader: Mezuru

The ratio seems to be approximately 3 males to 2 females.

Link to publication in Scopus. Alone we are rare. Investigational Therapies Information on current clinical trials is posted on the Internet at www. Current treatments, which can be used individually or in combination, include steroids, nutritional therapy fluid, electrolyte, protein and vitamin supplementation and, if necessary, an elemental diet or parenteral nutrition5-aminosalicylate acid, histamine H2 receptor antagonists, anti-tumor necrosis factor alpha agents, immunomodulators, and eradication of Helicobacter pylori.

Frequent endoscopic surveillance will be continued given high rates of concomitant colorectal and gastric cancer reported in this patient population. Based on these clinical, endoscopic, and histopathologic features, a diagnosis of Cronkhite-Canada Syndrome was made.

The polyps have hamartomatous features, meaning they contain mucus and are inflamed within an intact surface. Gastrointestinal stromal tumor Krukenberg tumor metastatic. N2 – Cronkhite-Canada syndrome CCS is a noninherited condition, associated with high morbidity, and characterized by gastrointestinal inflammatory polyposis, alopecia, onychodystrophy, hyperpigmentation, and diarrhea.

Helicobacter organisms were not identified in gastric or duodenal specimens.

AB – Cronkhite-Canada syndrome CCS is a noninherited condition, associated with high morbidity, and characterized by gastrointestinal inflammatory polyposis, alopecia, onychodystrophy, hyperpigmentation, and diarrhea.

Table of Contents Alerts. Squamous cell carcinoma Adenocarcinoma. CCS is believed secondary to immune dysregulation; however, the underlying etiology remains to be determined. Cronkhite-Canada syndrome CCS is a noninherited condition, associated with high morbidity, and characterized by gastrointestinal inflammatory polyposis, alopecia, onychodystrophy, hyperpigmentation, and diarrhea.


In this chapter, we provide a succinct overview of the history, etiology, risk factors, clinical manifestations, diagnosis, and management of CCS. Serum electrolytes platelet count, white count, renal, liver enzyme and function tests, lipase and total protein, serum immunoglobulins, CRP, and TSH were normal.

Cronkhite–Canada syndrome – Wikipedia

There were no extended nutrient deficiencies with lead, copper, zinc, B12, or iron. 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 syndroe. Self-skin examination New smartphone apps to check your skin Learn more Sponsored content. Histologically, the duodenal polyps showed edematous mucosa with variably dilated and branching glands, foci of gastric foveolar metaplasia, and blunted or absent intestinal villi.

The clinical course of CCS is progressive with occasional spontaneous remissions and frequent relapses, and with rare progression to malignancy. Laboratory values were within normal range.

Worldwide, over cases have been reported in the past 50 years, primarily in Japan but also in the United States and other countries. If you have any concerns with your skin or its cronjhite, see a dermatologist for advice. Springer International Publishing, There are usually large numbers of polyps in the digestive cronkhitte, most often sparing the esophagus. Patients with CCS can also have coexisting autoimmune disorders, where the body develops antibodies against an organ, thereby attacking itself, e.

Differential diagnosis includes familial adenomatous polyposis, hyperplastic polyposis syndrome, cap crlnkhite, juvenile polyposis syndrome, Peutz-Jeghers syndrome and Cowden syndrome see these termsas well as lipomatous polyposis, inflammatory polyposis and lymphomatous polyposis. The rest canadq the physical exam was unremarkable. Home About Us Advertise Amazon.

Despite adequate enteral caloric intake and immunosuppression, the patient continued to experience progressive weight loss, failure to thrive, and ongoing diarrhea C. Updated 14 January Cronkhite-Canada syndrome CCS is an extremely rare disease characterized by various intestinal polyps, loss of taste, hair loss, and nail cronkyite problems. Aggressive nutritional support in conjunction with immunosuppression has been used previously with inconsistent results. Etiology The pathogenesis of CCS is still elusive but an immune-mediated process has been proposed and this hypothesis is supported by the 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.


Severe rectal bleeding can cause anemia and episodes of recurring, severe abdominal pain.

Rare Disease Database

Hepatocellular adenoma Cavernous hemangioma hyperplasia: This condition was first described by Cronkhite and Canada inand the incidence is now estimated to be one per million persons per year [ 3 ].

Physical examination showed hair regrowth on the scalp, abdomen, and axillary and pubic regions in addition with improved proximal nail bed health. Page views in The foveolar glands were elongated, irregular, and focally dilated. The patient cznada noted an improved sense of taste. The abdomen was scaphoid and nontender with no hepatosplenomegaly. Click here for patient related inquiries.

Cronkhite-Canada Syndrome: Sustained Clinical Response with Anti-TNF Therapy

Familial adenomatous polyposis FAP is a rare inherited cancer predisposition syndrome characterized by hundreds to thousands of precancerous colorectal polyps adenomatous polyps appearing on average at age 16 years. Based on a successful recent case report, off-label infliximab was employed [ 6 cronkhte.

Summary and related texts. Pain management may also be required. Int J Surg Pathol. Medical history ceonkhite notable for prostate cancer curatively treated ingout, a remote transient ischemic attack, osteoarthritis, and bilateral cataracts. Views Read Edit View history.