Cronkhite-Canada Syndrome (CCS)—A Rare Case Report

Chakrabarti, Subrata

doi:10.7860/jcdr/2015/11919.5700

Cited by 16 publications

(10 citation statements)

References 14 publications

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“…Also due to the rarity of illness, optimal screening protocols have not been developed. Annual endoscopic surveillance for possible carcinoma screening and investigation for detection of SIBO should be considered to improve mortality and morbidity [ 3 ].…”

Section: Discussionmentioning

confidence: 99%

“…Polyps are found throughout the gastrointestinal tract with characteristic sparing of the esophagus [ 2 ]. The etiology of CCS is still obscure, but an autoimmune process has been suggested [ 3 ]. Approximately 450 cases of CCS have been reported worldwide [ 3 ].…”

Section: Introductionmentioning

confidence: 99%

“…The etiology of CCS is still obscure, but an autoimmune process has been suggested [ 3 ]. Approximately 450 cases of CCS have been reported worldwide [ 3 ]. Patients of European or Asian descent are most frequently affected and 75% of reports came from Japan [ 4 ].…”

Section: Introductionmentioning

confidence: 99%

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Cronkhite-Canada Syndrome: A Rare Cause of Chronic Diarrhoea in a Young Man

Bandyopadhyay

Hajra

Ganesan

et al. 2016

Case Reports in Medicine

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A young Indian man presented with nine-month history of chronic diarrhea, occasionally mixed with blood and intermittent colicky abdominal pain. He also complained of generalized body swelling for the last three months. On examination, he had diffuse hyperpigmentation of the skin and dystrophic nail changes. Upper and lower gastrointestinal endoscopy revealed multiple sessile polyps in the stomach, small bowel, and colon and rectum. Biopsy of polyps showed adenomatous changes with stromal edema and dilated glands. Cronkhite-Canada syndrome (CCS) was diagnosed and treated with glucocorticoids and enteral nutritional supplementation. There was an associated small intestinal bacterial overgrowth (SIBO) and stool was positive for clostridium difficile toxin. After 12 weeks of treatment, the patient achieved remission. Close correlation with clinical findings, including pertinent ectodermal abnormalities, endoscopic studies, and careful examination of biopsies will ensure a timely and correct diagnosis of CCS.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Cronkhite-Canada Syndrome: A Rare Cause of Chronic Diarrhoea in a Young Man

Bandyopadhyay

Hajra

Ganesan

et al. 2016

Case Reports in Medicine

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“…The mean age of onset is estimated to be in the sixth decade, with a slight male predominance 3:2 in ratio [1][2][3]. However, early onset in a young man in his age 26 had been reported [4].…”

Section: Discussionmentioning

confidence: 99%

Cronkhite-Canada syndrome: A rare case report and literature review

Lee¹,

Yang²,

Wu³

et al. 2018

biomedicalresearch

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Cronkhite-Canada Syndrome (CCS) is a rare, non-hereditary disease characterized by gastrointestinal polyposis associated with diarrhea and epidermal manifestations, such as hyperpigmentation, alopecia, and onychodystrophy. The pathogenesis of CCS is still not well understood but it probably involved autoimmune mechanism. Herein, we here report a case of steroid-responsive CCS in a male patient whose polyps presented with IgG-4 positive plasma cells. Keywords:Cronkhite-Canada syndrome, Hyperpigmentation, Autoimmune mechanism.Accepted on June 25, 2018 Case Report A 66 y old male farmer with no medical history of any systemic diseases had a 2 y history of non-bloody watery diarrhea associated with weight loss (more than 10 kg within 6 months). He defecated 6 to 8 times daily. He received upper and lower endoscopic examination, abdominal computer tomography, and small bowel barium series in the other hospitals, but there was no definite diagnosis. Endoscopic examinations revealed polyposis in both the stomach and colon. Pathology of the polyps showed inflammatory change without malignant change. Clinical symptoms were not alleviated after long-term proton pump inhibitor and mesalazine therapy. He had no family history of polyposis, malignancy of gastrointestinal tract, and he did not have fever and abdominal pain.His vital signs were normal on examination. General skin hyperpigmentation and dystrophic nails were found (Figure 1), but no oral pigmentation. Laboratory results revealed low level of serum albumin (2.7 g/dL, range: 3.8-5.3 g/dL) and cholesterol (100 mg/dL, range: 122-200 mg/dL). The erythrocyte sedimentation rate was elevated (42 mm/h, range: <15 mm/h), and the C reaction protein slightly increased (0.847 mg/dL, range: <0.3 mg/dL). Serum IgG was within normal range, and anti-nuclear antibody was negative. Gastroesophageal endoscopy found diffuse mucosal erythematous gastric mucosa with fold thickening and numerous semi-pedunculaed and sessile polyps with variable size at the cardia, body, antrum, and duodenal bulb with the exception of esophagus. Ileo-colonoscopic examination showed multiple erythematous sessile and semi-pedunculated polyps with variable size from ileocecal valve, cecum, to the left side colon (Figures 2A and 2B). Multiple EMR and polypectomy from the gastric and colon polyps showed retention polyps with cystically dilated glands filled with mucus in an edematous and inflamed lamina propria mainly composed of plasma cells, neutrophils, and eosinophils. IgG4-positive plasma cells were seen with uneven distribution (Figure 3). Focally, the number of IgG4-positive plasma cells was up to 30 in one high power field. The IgG4 to IgG-positive cell number ratio was around 40%. Helicobacter pylori was negative. Combining the polyposis, ectodermal manifestations, and clinical symptoms, he was diagnosed with Crokhite-Canada syndrome. The patient was treated with daily oral prednisolone 30 mg per day and then tapered over 3 months. The frequency of diarrhea was reduced and he gradually ga...

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“…For patients with CCS, diarrhea is the most common initial symptom, which may develop to substantially watery diarrhea, followed with symptoms of malabsorption, including weakness, anemia, weight loss, edema and dysgeusia (17). Subsequent endoscopic and radiological evaluation may reveal sessile polyps throughout the GI tract (18).…”

Section: Discussionmentioning

confidence: 99%

Cronkhite-Canada syndrome: A case report

Rubio

Björk

2018

Journal of the American Academy of Dermatology

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Abstract. Cronkhite-Canada syndrome (CCS) is a rare non-inherited condition characterized by gastrointestinal (GI) hamartomatous polyposis, alopecia, onychodystrophy, hyperpigmentation, weight loss and diarrhea. The etiology is most likely autoimmune and diagnosis is based on patient history, physical examination, endoscopic findings of GI polyposis and histology. The disease is very rare; thus far more than 500 cases of CCS have been reported globally. A 58-years-old male with CCS was reported in the present case study. The patient experienced a history of diarrhea and hematochezia for 4 months, with abdominal pain for 1 month and additional nail and toenail loss for half a month. The clinical, endoscopic and histological data confirmed the diagnosis.

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Cronkhite-Canada Syndrome (CCS)—A Rare Case Report

Cited by 16 publications

References 14 publications

Cronkhite-Canada Syndrome: A Rare Cause of Chronic Diarrhoea in a Young Man

Cronkhite-Canada Syndrome: A Rare Cause of Chronic Diarrhoea in a Young Man

Cronkhite-Canada syndrome: A rare case report and literature review

Cronkhite-Canada syndrome: A case report

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