Clinco-pathologic Features of Alopecia in a Patient of Cronkhite-Canada Syndrome / 대한피부과학회지

Although hair loss in Cronkhite-Canada syndrome (CCS) developed frequently, its exact mechanism has not been elucidated. Thus, we attempted to investigate the histopathologic features of hair loss with scalp biopsy in one case of CSS. The patient complained of persistent diarrhea and diffuse hair loss of the scalp 1 month prior to the hospital visit. CCS has been already diagnosed through colonoscopy and medical examination. Scalp biopsy was performed to evaluate hair loss of the patient, and pathologic examination showed increased telogen hairs (anagen to telogen is 7:8) without specific dermal inflammation and miniaturization of hair follicles. The patient was treated with topical and systemic steroids, and hair loss has almost completely recovered in 5 months. In this case, we investigated the clinical and pathological features of hair loss through scalp biopsy in one case of CCS and reported them with a review of the literature.

Clinicopathologic features of Cronkhite-Canada syndrome and the significance of IgG4-positive plasma cells infiltration / 中华病理学杂志

Objective@#To analyze the clinical and pathological features of Cronkhite-Canada syndrome (CCS), and to investigate the significance of IgG4 positive plasma cell infiltration.@*Methods@#Clinical presentations, endoscopic appearances and morphological features of 18 patients diagnosed with CCS at Peking Union Medical College Hospital during 2000-2016 were included in the study.There were 11 male and 7 female patients. IgG4 and IgG immunohistochemical stains were performed in total of 55 biopsies from the patients (36 polyps, 10 adenoma and 9 surrounding mucosa) and a control group of 58 cases (19 colonic mucosa, 7 colonic hyperplastic polyps, 9 inflammatory bowel disease and 23 adenoma).@*Results@#Average age of CCS was 59 years (range 47-69 years) and the male to female ratio was 11∶7. All patients had at least one ectoderm lesion. Fourteen cases had testicular abnormalities. Digestive tract symptoms were encountered in most cases. Four patients had a history of connective tissue disease. Endoscopically, multiple polyps were found to involve entire gastrointestinal tract except the esophagus. Morphologically, CCS polyps were characterized by prominent mucosal edema, mild to moderate inflammation, glands hyperplasia and cystic dilatation. Ten cases had colonic adenoma and one case had rectal adenocarcinoma. While none of the cases reached the diagnostic criteria of IgG4 disease, the positive rate of IgG4 positive plasma cells in adenoma of CCS was significantly higher than that in polyps and surrounding mucosa of CCS (60.0%∶13.9%∶0, P<0.01). The positive rate of IgG4 positive plasma cells of adenoma and polyp of CCS had an increased tendency compared to that of adenoma and polyp of control group (60.0%∶34.8%; 13.9%∶0).@*Conclusion@#The findings in the study do not support that CCS is a variant of IgG4 disease, although IgG4 positive plasma cells may be involved in the pathogenesis of CCS, which may be explored in future investigations.

A Case of Cronkhite-Canada Syndrome Showing Spontaneous Remission

Cronkhite-Canada syndrome (CCS) is a rare, noninherited gastrointestinal polyposis syndrome associated with ectodermal changes such as alopecia, nail dystrophy, and cutaneous hyperpigmentation. The etiology and pathogenesis of CCS are not known, but diarrhea, malnutrition, gastrointestinal bleeding, and infection may occur in the affected patient; moreover, this condition could be fatal. However, previous reports have described several cases of spontaneous remission. We report a 60-year-old man who was incidentally found to have colonic polyposis, alopecia, and hypogeusia and was diagnosed to have CCS. However, this patient experienced spontaneous remission, including regrowth of body hair and alleviation of bowel inflammation, without any specific medications such as steroids, antibiotics, or proton pump inhibitors.

A Case of Cronkhite-Canada Syndrome with Esophageal Candidiasis

Cronkhite-Cadana syndrome is a rare non-familial disease. This syndrome is characterized by multiple hamartomatous polyps on the entire gastrointestinal tract except esophagus, nail dystrophy, alopecia and hyperpigmentation. Taste disturbance, abdominal pain, diarrhea and weight loss are common symptoms of it. The pathogenesis and causes of Cronkhite-Canada syndrome remain unknown until now. Although various treatment strategies including steroid therapy have been tried, their prognosis is poor. We report a 68 years old man who were diagnosed Cronkhite-Canada syndrome with esophageal candidiasis. After using combination of steroids and anti-fungal drugs, both Cronkhite-Canada syndrome and esophageal candidiasis were cured.

Cronkhite-Canada Syndrome That Developed in a Patient Taking Levothyroxine Sodium after Total Thyroidectomy / 대한피부과학회지

Cronkhite-Canada Syndrome (CCS) is a rare, non-inherited hamartomatous polyposis syndrome. It is characterized by diffuse gastrointestinal polyposis, diarrhea, weight loss and unique ectodermal manifestations such as alopecia, onychodystrophy and cutaneous hyperpigmentation. The etiology of CCS remains unknown, but an autoimmue contribution to the pathogenesis has been postulated. We report here on a 79-year-old male who has been diagnosed with CCS. He had been taking levothyroxine sodium after total thyroidectomy. To the best of our knowledge, this is the first case report of CCS in association with hypothyroidism and supplementation with levothyroxine sodium in the Korean medical literature.

A Case of Cronkhite-Canada Syndrome Conducted with Capsule Endoscopy of Small Intestine / 대한소화기내시경학회지

Cronkhite-Canada syndrome is a very rare syndrome. This non-familial hamartomatous polyposis syndrome is characterized by multiple polyps on the entire gastrointestinal tract, nail dystrophy, skin pigmentation and systemic alopecia. The courses of this syndrome could be classified into five types according to clinical symptoms; diarrhea, taste disturbance, xerostomia, abdominal pain and alopecia. Cronkhite-Canada syndrome has a high mortality rate up to 45~60% due to nutritional absorption disturbance, hypoalbuminemia, recurrent infection, sepsis, heart failure and gastrointestinal bleeding. A pathogenesis of Cronkhite-Canada syndrome is still unknown, and only conservative treatment is available. We diagnosed a 55 years-old female with Cronkhite-Canada syndrome based on the clinical symptoms of nail change, taste disturbance and alopecia, and the histologic finding of polyps in the entire gastrointestinal tract; these polyps were found in the stomach, small intestine and large intestine via capsule endoscopy. We report on this case and we review the relevant medical literature.

A Case of Cronkhite-Canada Syndrome Showing Resolution with Helicobactor pylori Eradication and Omeprazole / 대한소화기학회지

We describe a 58-year-old woman who was incidentally found to have gastric and colonic polyposis, hypoalbuminemia, cutaneous hyperpigmentation and onychodystrophy (Cronkhite-Canada syndrome). Histology of polyps from the stomach showed features of juvenile or retention type (hamartomatous) polyps with Helicobacter pylori (H. pylori) infection. The large pedunculated colonic polyps showed hamartomatous polyps with adenomatous component and polypectomy was performed. After the treatment with H. pylori eradication and omeprazole, the gastric polyposis, hypoalbuminemia and anemia regressed, and endoscopic polypectomy of gastric polyps were performed. After the continuous use of omeprazole for 14 months, the patient showed complete resolution of clinical features of Cronkhite-Canada syndome. The experience of this case suggests that eradication of H. pylori and proton pump inhibitor treatment might be considered in patients with gastric polyposis combined with Cronkhite-Canada syndome.

A Case of Cronkhite-Canada Syndrome with a Remission to Steroid Therapy / 대한소화기내시경학회지

Cronkhite-Canada syndrome is characterized by generalized gastrointestinal polyposis, ectodermal changes, and the eventual development of diarrhea and weight loss. The pathogenesis of the disease is unknown, and there is no established therapy. The disease has a poor prognosis because of malnutrition resulting from altered absorption in the gastrointestinal tract. We experienced a case of a 56-year-old female with Cronkhite-Canada syndrome. After enteral nutrition and administration of prednisolone for 3 months, clinical improvement was noted with cessation of diarrhea, increased serum protein, disappearance of pigmentation, and regrowth of the scalp hair, finger-and toenails. Endoscopy showed resolution of the gastrointestinal polyposis. So we report here a case of diffuse gastrointestinal polyposis which has been in remission with steroid therapy.

A Case of Cronkhite-Canada Syndrome / 대한피부과학회지

The Cronkhite-Canada syndrome is a rare, non-neoplastic, non-hereditary condition which consists of gastrointestinal polyposis associated with alopecia, onychodystrophy and hyperpigmentation of the skin. It usually encountered with severe diarrhea, weight loss and other malabsorption symptoms. The etiology of the condition is unknown. Usually, this syndrome is associated with a poor prognosis due to the effect of malabsorption and generalized debility. We report a case of this syndrome showing unusually rapid improvement after 3-month following only conservative treatment. To our knowledge, this is the first case report of Cronkhite-Canada syndrome in the Karean dermatologic literature.