Topic > Cronkhite-Canada syndrome (ccs) - a rare case

Index IntroductionCase reportLaboratoryEndoscopyDiscussionIntroductionCronkhite-Canada syndrome (CCS) is a sporadic condition involving tissues arising from the ectodermal germ cell layer. It is characterized by gastrointestinal hamartomatous polyposis, diarrhea, hypoproteinemia and skin manifestations including alopecia, onychodystrophy, hyperpigmentation. Other symptoms such as disguisea, hypoguisea and xerostomia due to zinc and cobalt deficiency have been reported in some patients. Its incidence is low but mortality is high. Only 500 cases of CCS have been reported worldwide, with an overall mortality rate of approximately 60%. The main age of presentation is 59 years with a slight male predominance in the ratio of 3:2, however, most cases identified in the age above 50 years. Say no to plagiarism. Get a tailor-made essay on "Why Violent Video Games Shouldn't Be Banned"? Get an original essayThe first case of Cronkhite-Canada syndrome was reported in 1955 by the internist Leonard Wolsey Cronkhite and the radiologist Wilma Jeanne Canada in the New England Journal of Medicine, the etiopathogenesis of this disease is not yet fully understood, but they exist two schools of thought: one which supports the immunological mechanism, which is most believed in, and the other which supports the infectious mechanism. The latter was also taken into consideration due to the histopathological mechanism: the results of the biopsy of gastrointestinal polyps showed an infiltration of inflammatory cells with mononuclear cells and eosinophils. While CCS polyps are infiltrated with IgG4 plasma cells; elevated antinuclear antibodies; the decreased disease manifestation when treated with corticosteroids and the association with other autoimmune diseases such as hypothyroidism, systemic lupus erythematosus, rheumatoid arthritis, and scleroderma strongly support the autoimmune pathophysiology of this disease. The only hypothesized risk factors for CCS include mental and physical stress. Case Report A 44 year old Indian male residing in Canada for the last 16 years with a history of gout for 7 years presented with complaints of chronic diarrhoea, intermittent bloody stools, nausea, abdominal pain, weight loss for 2 months, occasionally took alcohol in the last 10 years. He was taking proton pump inhibitors for chronic gastritis, allopurinol for gout, ibuprofen for the symptomatic relief of gouty pain and after a week of ibuprofen he spontaneously developed diarrhea which did not resolve with drugs with increasing frequency up to 10 times a day, with his hair initially grays from the pubic to the axillary hair, progressing to the mustache and beard with the scalp as the last site affected. She initially had intermittent abdominal cramps that developed into several attacks of abdominal pain. He began to lose all his hair, including his nasal hair, causing continuous nasal discharge and dry nasal mucosa. and developed brittle nails that started falling out. During this period he suffered from severe malnutrition and lost almost 4 kg of weight in 3 weeks. At his first hospitalization, a gastrointestinal disease was suspected, so he was prescribed pantoprazole and antibiotics after x-rays and blood tests. After further investigation, he underwent an esophagogastroduodenoscopy with biopsy which revealed small sessile polyps throughout the digestive tract except the esophagus. Corticosteroid treatment was soon started, keeping in mind a possible immune response leading to polyp formation. In the following 5 weeks he further lost 6 kg of weight with loss of taste sensation. He was subsequently subjected tovideocolonoscopy during which a biopsy of colon and stomach polyps was performed for immunohistological investigations. On physical examination he was of fair build, poorly nourished, weighed 68 kg, had no eyebrows, baldness, onychodystrophy with loss of fingers and toenails. Gouty swelling was present on the right finger. There were hyperpigmented oval patches measuring 5 mm to 15 mm in diameter all over the palms of the hands and soles of the feet. His palms were sweaty and there was some hyperpigmentation scattered across different parts of his body. Physical examination also showed bilateral symmetric edema in the lower extremities. There were no other significant findings. Symptomatic treatment initially included corticosteroids, analgesics, and broad-spectrum antibiotics. Nutritional support was provided with multivitamin tablets, calcium and iron. PPIs have been discontinued. A high protein diet was recommended. It reduced the symptoms but not effectively. Then in India, he stopped all medications and started Ayurvedic powder with diet treatment. The Ayurvedic powder was a mixture of Aegle marmelos (Indian bael) dried fruit, Zingiber officinale (dried ginger), Gymnema sylvestre leaf extract in the ratio of 1:1:2 respectively. The daily diet consisted only of turmeric milk, broken wheat and puffed rice. Calcium and iron folic acid tablets were supplemented. This diet was followed for six weeks. By the end of the 6th week only 2 episodes of bloody diarrhea occurred, stool frequency decreased to 5-6 times from 10 times a day. After six weeks, khichri, an Indian dish made from cracked wheat and lentils was added to his diet. The Ayurvedic powder has been replaced by Ayurvedic tablets consisting of Holarrhena antidysenterica bark extracts, Plumbago zeylanica root extracts, Piper longum root extracts, Bryophyllum pinnatum leaf extracts and Cinnamomum camphora bark extracts. Six weeks later he started growing white hair on his eyebrows, mustache and pubic area. Stool frequency decreased to 3-4 times per day. The hair began to blacken, the weight increased by 2 kg and finally new nails began to grow. A CT scan was also performed at the end of 12 weeks from the start of diet-based treatment which supported the improvement of the pathology. He has currently stopped all Ayurvedic medicines and is taking Mesalazine 1200 mg, four times a day. Laboratory His eosinophil count was 7.4% (normal range: 0.0 - 6) with an absolute count of 0.58 x 10^3/μl (normal range: 0 - 0.5), his hemoglobin was 12.5 g/dl (normal range 13 – 17), had an average corpuscular hemoglobin of 26 pq (normal range 27-32), average corpuscular hemoconcentration of 26.5 g/dl (normal range 31.5 – 34.5) Red blood cell distribution width – Sd of 53.6 fL (normal range 39-46) Red blood cell distribution width – CV of 14.6% (normal range 11.6-14), 25-OH vitamin Total D of 6.5 ng/ml, calcium levels of 8.09 mg/dl (normal 8.8 – 10.6, uric acid levels of 9.4 (normal range 3.7 – 9.2), estimated glomerular filtration rate of 81 (between 60 – 89 indicates a slight decrease. Endoscopy Esophagogastroduodenoscopy revealed polyps with atrophy of the stomach villi and a normal esophagus. Some hemorrhagic foci were present on the internal surface of the stomach. In the duodenum, the rugal folds were thickened and had a brownish color. Multiple polyps have been observed in the corpus and antrum of the stomach; the first, second and third parts of the duodenum. Colonoscopy revealed multiple polyps and inflammation throughout the colon..