Cronkhite-Canada syndrome (CCS) is a rare nonhereditary disorder characterized by gastrointestinal polyps and protein-losing enteropathy. While an increasing number of CCS cases have been reported worldwide, no documented cases involving pregnant patients could be found. Consequently, optimal management strategies for CCS during the preconception period and pregnancy remain unclear., The present report concerns the case of a 36-year-old woman with steroid-refractory CCS stabilized with gastrointestinal surgeries and infliximab, an anti-tumor necrosis factor-α (TNF-α) agent, who became pregnant. Infliximab was continued throughout pregnancy and postpartum. Despite persistent hypoalbuminemia, sh delivered a healthy infant weighing 2518 g vaginally at 38 weeks and 2 days without CCS exacerbation. Both the patient and her infant experienced an uneventful postpartum course. This case suggests that maintaining disease control with anti-TNF-α therapy in pregnant patients with CCS may contribute to optimizing maternal and neonatal outcomes.

Cronkhite-Canada syndrome (CCS) is a rare nonhereditary disorder characterized by gastrointestinal polyps and ectodermal manifestations, such as alopecia and nail dystrophy. This report highlights the diagnostic and therapeutic challenges of CCS and contributes to the global understanding of the syndrome. A 50-year-old woman presented with persistent anorexia, fatigue, abdominal discomfort, alopecia, and onychodystrophy. Diagnostic evaluation revealed characteristic endoscopic findings of gastrointestinal polyps and histopathological features consistent with CCS. The patient was treated with glucocorticoids, resulting in clinical and nutritional improvement. Nutritional support and immunosuppressive agents may serve as adjunct therapies, but further research is needed to establish definitive guidelines. This case underscores the importance of thorough endoscopic and histopathological evaluations in rare syndromes like CCS. It also emphasizes the significance of early diagnosis and a multidisciplinary approach to disease management.

Cronkhite-Canada syndrome (CCS) is a non-hereditary disorder characterized by non-neoplastic hamartomatous gastrointestinal polyposis, hair loss, nail atrophy, hyperpigmentation, and diarrhea. While the relationship between CCS and nephritis remains unclear, seven cases of nephritis complicated by CCS have been reported to date, all of which were membranous nephropathy (MN). A 57-year-old man presented with taste disturbance, hair loss, nail plate atrophy, skin pigmentation, and frequent diarrhea. Endoscopic findings showed multiple polyposis of the stomach and large intestine. Given the above, he was diagnosed with CCS. The symptoms gradually improved with prednisolone treatment, although urinary protein and hypoproteinemia appeared during the tapering of prednisolone. He was diagnosed with MN using a renal biopsy, and immunofluorescence microscopy with IgG subclass staining showed predominantly diffuse granular capillary wall staining of IgG4. The cause of secondary MN was not found, including malignant tumors. Nephrotic-range proteinuria persisted despite treatment with prednisolone and cyclosporine. Additional treatment with mizoribine resulted in incomplete remission type 1 of nephrotic syndrome, suggesting that mizoribine may be a treatment option for patients with CCS with steroid-resistant MN. Considering a high prevalence of hypoproteinemia due to chronic diarrhea and protein-losing enteropathy in patients with CCS, proteinuria might be overlooked; thus, follow-up urinalysis would be recommended in patients with CCS.

Cronkhite-Canada syndrome (CCS) is a nonhereditary, rare polyposis condition, first documented by Cronkhite and Canada in 1955. The primary distinct features of this syndrome include ectodermal abnormalities and diffuse gastrointestinal polyp changes accompanied by protein loss. The primary clinical manifestations of CCS include hair loss, excessive pigmentation of the skin, and malnourishment of fingernails or toenails. Other notable symptoms include weight loss, protein-losing enteropathy, diarrhea, abdominal pain, nausea, vomiting, taste abnormalities, and atrophic glossitis, which predominantly occur in middle-aged and older males. CCS is characterized by an extremely rare, nonfamilial hamartomatous polyposis syndrome, in which polyps are distributed in the stomach and colon (90%), small intestine(80%), and rectum (67%), while sparing the esophagus.

This report describes a 72-year-old female, initially treated for intestinal obstruction, followed by a small intestine resection. She reported diarrhea, emaciation, and loss of appetite across various hospitals.

Endoscopic examination of the stomach and colon, plus capsule endoscopy, revealed multiple polyps throughout her gastrointestinal tract, except in the esophagus.

Treatment included hormones with antiallergic medication, acid-suppressing drugs, salicylates, and nutritional support with zinc sulfate, adding trace elements and amino acids.

posttreatment, the patient demonstrated significant improvement in appetite and taste. Atrophic glossitis, upper limb pigmentation, and frequency of diarrhea also notably decreased. reexamination through endoscopy after 3 months of treatment revealed a substantial decrease in the number and size of gastrointestinal polyps.

In this case, from the lower esophageal sphincter to the rectum, there is an increasing trend of eosinophil and mast cell infiltration. These lesions can cause a positive IgG result. Pathological analysis indicates that the extent and severity of lesions in the middle and lower gastrointestinal tract are more substantial than in the upper tract. During treatment, endoscopic observations reveal that lesions in the middle and lower tract tend to resolve faster than those in the upper tract. Hormone therapy has demonstrated significant efficacy in treating this disease. Early treatment and regular follow-up for this disease can reduce the risk of cancerous changes and related complications.

We present a case of Cronkhite-Canada syndrome in a patient with decompensated cirrhosis who had successful induction of remission with nutritional supplementation alone. We propose that early institution of high-protein, high-energy enteral supplementation should be offered to all patients, especially those with compelling contraindications to immunosuppression.

Cronkhite-Canada syndrome is a rare gastrointestinal polyposis syndrome with distinctive clinical features and endoscopic findings. Diagnosis can be challenging without suspicion, and the disease carries high mortality due to complications such as infection, gastrointestinal bleeding, and malignancies. This paper presents two cases of Cronkhite-Canada syndrome occurring after coronavirus disease 2019 (COVID-19) mRNA vaccination. Both cases exhibited typical clinical findings, including hypogeusia, onychodystrophy, alopecia, and weight loss. Typical polyposis in the gastrointestinal tract was confirmed through endoscopies. As symptomatic treatment did not improve the symptoms, corticosteroids were administered, and symptoms and laboratory test results improved immediately. The patients improved upon corticosteroids tapering. These cases illustrate typical presentations of Cronkhite-Canada syndrome and the course of the disease following corticosteroid treatment. Additionally, they suggest the possibility that Cronkhite-Canada syndrome may be triggered by COVID-19 mRNA vaccination.

Although prednisolone treatment is effective in Cronkhite-Canada syndrome (CCS), its mechanisms of action are poorly understood. We performed analyses of cytokine expression and fecal microbiota in a patient with the concurrent occurrence of CCS and rectal cancer, in whom regression of polyposis was achieved by prednisolone. Regression of CCS polyps was accompanied by downregulation of proinflammatory cytokine expression and alterations in microbiota composition; a decrease in Bacteroides fragilis and Peptostreptococcus anaerobius with the promotion of inflammation. We could not completely exclude the possibility that alterations in fecal microbiota composition might be influenced by the presence of advanced cancer. However, this case suggests that the administration of PSL might lead to the regression of CCS polyps through alterations in gut microbiota composition and suppression of proinflammatory cytokine responses.

Cronkhite-Canada syndrome (CCS) is a rare, noninherited disease characterized by gastrointestinal polyposis with diarrhea and ectodermal abnormalities. CCS polyps are distributed through the whole digestive tract, and they are common in the stomach and colon but very uncommon in the esophagus.

Here, we present a case of a 63-year-old man with skin hyperpigmentation accompanied by diarrhea, alopecia, and loss of his fingernails. Laboratory data indicated anemia, hypoalbuminemia, hypocalcemia, hypokalemia, and positive fecal occult blood. Endoscopy showed numerous polyps scattered throughout the digestive tract, including the esophagus. He was treated with nutritional support and glucocorticoids with remission of his symptoms.

Comprehensive treatment led by hormonal therapy can result in partial or full remission of clinical symptoms. Treatment should be individualized for each patient according to their therapy response. Surveillance endoscopy is necessary for assessing mucosal disease activity and detecting malignant transformation.

Cronkhite-Canada syndrome (CCS) is a rare sporadic polyposis syndrome that presents with gastrointestinal and ectodermal symptoms in addition to nutritional deficiencies. CCS combined with hypothyroidism is an even rarer condition, with no standard treatment guidelines.

The present study described 2 patients with CCS: A 67-year-old woman with concomitant hypothyroidism and 68-year-old man treated with endoscopic mucosal resection (EMR). Both patients had multiple gastrointestinal symptoms and ectodermal changes, along with multiple gastrointestinal polyps. Microscopic examination showed that the mucosa in both patients was hyperemic and edematous, with pathologic examination showing distorted, atrophic, and dilated glands. Patient 1 had concomitant hypothyroidism and was treated with levothyroxine. Due to her self-reduction of hormone dose, her disease relapsed. Patient 2 underwent EMR, but refused further hormonal or biological treatments. Subsequently, he was treated with an oral Chinese medical preparation.

Pharmacotherapy can induce and maintain remission in CCS patients, with adjuvant EMR, long-term follow-up, and endoscopic surveillance being necessary.

Cronkhite-Canada syndrome (CCS) is considered a relentlessly progressive disease with high mortality rates. Although disease understanding and treatment options have greatly improved, the prognosis from these advancements has not been well documented. This study aimed to evaluate treatment outcomes and overall survival of CCS.

Seventeen patients who were diagnosed and treated over a 20-year period at Mayo Clinic (Rochester, Minnesota) were included. Data were abstracted, which included clinical and endoscopic manifestations, treatment course, and survival outcomes.

The median (interquartile range) duration of follow-up was 8.3 (3.7-15.8) years. All patients received an initial prednisone dose equivalence of 30-80 mg daily, and five patients required steroids at the time of the last follow-up. Twelve patients trialed thiopurine therapy, and ten patients continued with a thiopurine until the last follow-up. Fifteen patients achieved clinical complete remission, and eleven patients achieved endoscopic complete remission after pharmacotherapy initiation. Seven patients required gastrointestinal surgeries during their disease course. The 5-year overall survival was 93.3% (95% confidence interval (CI): 81.5-100%), and the 3-year relapse-free survival was 82.4% (95% CI: 66.1-100%).

The prognosis and overall survival of patients with CCS have markedly improved with advancement in disease understanding and therapies. Pharmacotherapy, including corticosteroids and immunomodulators, is effective in inducing and maintaining remission, and gastrointestinal surgery is commonly needed as an adjunct for managing CCS disease complications.

The hamartomatous polyposis syndromes are a set of clinically distinct disorders characterized by the occurrence of hamartomatous polyps in the gastrointestinal tract. These syndromes include juvenile polyposis syndrome, Peutz-Jeghers syndrome, and PTEN hamartoma tumor syndrome. Although each of the syndromes has distinct phenotypes, the hamartomatous polyps can be challenging to differentiate histologically. Additionally, each of these syndromes is associated with increased lifetime risks of gene-specific and organ-specific cancers, including those outside of the gastrointestinal tract. Germline pathogenic variants can be identified in a subset of individuals with these syndromes, which facilitates molecular diagnosis and subsequent gene-enabled management in the setting of genetic counseling. Although the malignant potential of hamartomatous polyps remains elusive, timely recognition of these syndromes is important and enables presymptomatic cancer surveillance and management before symptom exacerbation. Presently, there are no standard agents to prevent the development of polyps and cancers in the hamartomatous polyposis syndromes.

Cronkhite-Canada syndrome (CCS) is an acquired polyposis syndrome with gastrointestinal and extraintestinal manifestations. Given its rarity and lack of standard treatment, diagnosis and treatment are challenging. Steroid therapy and nutritional support are conventional treatments. There is no consensus on management of steroid-refractory cases. Here, we report the diagnosis and treatment course of a 54-year-old Asian male with CCS, whose initial treatment with prednisone 60 mg a day led to partial response and disease flare up during prednisone tapering. The use of infliximab and azathioprine led to promising remission of his symptoms.

Cronkhite-Canada syndrome is a rare sporadic polyposis syndrome that presents with dermatologic and neurologic symptoms in addition to nutritional deficiencies. It can mimic alternate pathologies, such as Menetrier disease, making adequate histologic sampling with deep snare biopsies necessary for tissue comparison. We present a case report of Cronkhite-Canada syndrome that demonstrates the importance of deep tissue sampling for adequate diagnosis and treatment initiation.

Gastrointestinal (GI) polyposis is a rare condition in GI diseases. To date about 500 cases of Cronkhite-Canada syndrome (CCS) have been reported worldwide.

We report a 60-year-old female patient who presented with dyspepsia, abdominal pain, and weight loss of 1-year duration. Her physical examination showed alopecia and onychodystrophy. Upper endoscopy revealed diffuse markedly thickened gastric mucosa involving the whole stomach with thickened gastric rugae and numerous polypoidal lesions. Histopathological examination showed marked hyperplasia of the foveolar glands with inflammatory cell infiltration. Endoscopic ultrasound showed a significantly hypertrophic mucosa and muscularis mucosa, while the submucosa and the muscularis propria were spared, favouring its benign nature. Colonoscopy showed multiple sessile polyps scattered at different parts of the colon. Histopathological examination revealed tubular adenomatous polyps with low-grade dysplasia. Differential diagnoses included CCS, Menterier disease (MD), other polyposis syndromes, lymphoma, amyloidosis, and gastric malignancies. The presence of alopecia, nail dystrophy, GI polyposis, markedly thickened gastric mucosa and folds, abdominal pain, weight loss, and marked foveolar gland hyperplasia; all was in favour of CCS. Lymphoma was excluded due to sparing of the muscularis propria. The presence of colonic polyps and antral and duodenal infiltration, and the absence of hypoproteinaemia decreased the possibility for MD.

The patient was diagnosed as having CCS.

Cronkhite-Canada syndrome (CCS) is a rare disease that is characterized by multiple gastrointestinal polyps and ectodermal abnormalities. This study aimed to improve the understanding of CCS by presenting our patient data.

Clinical features, treatment, and outcomes of four CCS patients at a single medical center were retrospectively analyzed.

The age of the patients ranged from 32 to 61 years (mean: 49.5 years), including three men and one woman. All the patients presented with gastrointestinal symptoms, ectodermal abnormalities, and multiple gastrointestinal polyps. Two patients showed abnormal immune indices. Three patients underwent magnetic resonance enterography, and the typical manifestations of small intestine involvement were diffuse wall thickening, high signal intensity on diffusion-weighted imaging, obvious enhancement, and multiple small nodular enhancements of the small intestine. The main histological manifestations were chronic inflammation and hyperplastic, adenomatoid, and hamartomatoid polyps. Eosinophilic infiltration was observed in two patients. One patient had rectal adenocarcinoma at the time of diagnosis. All the four patients received prednisone at a dose of 0.75-1 mg/kg/day, and had their gastrointestinal symptoms gradually resolved (including two with ectodermal abnormality and endoscopic remission). Two patients are currently receiving low-dose prednisone (2.5-5 mg/day) with no recurrence after a 1.5- and 6-year follow-up periods, respectively.

Magnetic resonance enterography has the potential to evaluate small-intestinal lesions in CCSs. Long-term therapy with low doses of prednisone may be beneficial in maintaining remission.

Crohn's disease is a chronic inflammatory disease that can affect any portion of the gastrointestinal tract. Associated symptoms can vary based on the severity of disease, extent of involvement, presence of extraintestinal manifestations, and development of complications. Diagnosis is based on a constellation of findings. Many diseases can mimic Crohn's disease and lead to diagnostic conundrums. These include entities associated with the gastrointestinal luminal tract, vascular disease, autoimmune processes, various infections, malignancies and complications, drug- or treatment-induced conditions, and genetic diseases. Careful consideration of possible causes is necessary to establish the correct diagnosis.

Cronkhite-Canada syndrome (CCS) is a rare non-hereditary disease of unknown etiology that is characterized by the appearance of multiple polyps in the entire gastrointestinal (GI) tract, except in the esophagus, with GI and non-GI symptoms. Various factors are associated with the pathogenesis of CCS. Immune dysregulation has been discussed as one of the pathogeneses of CCS, and dysbiosis of the gut microbiota can affect the immune system. Currently, standard treatment has not been established.

We present the treatment with fecal microbiota transplantation (FMT) in a 67-year-old male patient with steroid-refractory CCS who could not undergo anti-tumor necrosis factor-a treatment due to suspected tuberculosis infection.

FMT has recently attracted attention as a method of overcoming drug resistance through immunomodulatory effects through microbiome regulation. We collected the patient's stool samples before FMT and 8weeks after FMT.

We analyzed the microbiome composition of patients by sequencing the V3-V4 region of the 16s rRNA gene (Miseq). After FMT, the number of episodes of diarrhea and hypoalbuminemia were also corrected. The Chao 1 index after FMT, which was significantly higher than that of donors before FMT, changed to a similar level for donors after FMT. Fusobacterium nucleatum, Pyramidobacter piscolens, and Campylobacter concisus disappeared after FMT, suggesting the presence of an association between gut microbiota and CCS.

Furthermore, we provide the possibility that microbiome modulation by FMT could serve as a complementary treatment in patients with steroid-refractory CCS.

Cutaneous findings are commonly associated with underlying gastrointestinal disorders and, in many instances, may be the first manifestation. Many such syndromes have incomplete penetrance and variable expressivity, making them difficult to recognise. Skin manifestations may be an easily recognised feature of the underlying disorder. Most of these syndromes are hereditary but not all are associated with malignancies; either benign or premalignant extraintestinal lesions can be the initial manifestation. Some involve a single organ system, while others involve multiple organs of the gastrointestinal tract. In this review, we have focused on Lynch syndrome (hereditary nonpolyposis colon cancer and Muir-Torre syndrome), familial adenomatous polyposis, the hamartomatous polyposis syndromes that include Peutz-Jeghers syndrome and the PTEN hamartoma syndromes, which include Cowden syndrome and Bannayan-Riley-Ruvalcaba syndrome and, lastly, Cronkhite-Canada syndrome, which is not heritable. Some of these are associated with colorectal cancer, of which 15% are heritable. The majority are inherited in an autosomal dominant fashion. These syndromes are uncommon. However, because of the strong association with the cutaneous findings, early detection and screening may be possible and are key to decreasing the morbidity and mortality associated with them, for both the patient and family members. The clinical findings, epidemiological findings, underlying genetic alterations and pathological findings are reviewed.

This study aimed to investigate the clinical features and potential pathogenesis of a rare nonhereditary polyposis syndrome, Cronkhite-Canada syndrome (CCS).

Medical records of eight patients with CCS who were admitted to our hospital from January 2005 to November 2019 were reviewed. Transcriptome profiling was performed in one patient to investigate its difference between gastric polyp tissue and normal mucosa. Differentially expressed genes (DEGs) were determined for functional analysis. The expression of inhibin beta A (INHBA) was further assessed by using immunohistochemistry.

All patients presented with gastrointestinal polyposis, accompanied by diarrhea, skin hyperpigmentation, hair loss and nail dystrophy. Hyperplastic polyps were observed in seven patients, tubular adenoma in two, inflammatory polyps in one and hamartomatous polyps in one, respectively. All patients underwent comprehensive treatment and five achieved clinical remission. A total of 2107 DEGs, including 1265 upregulated and 842 downregulated, were found in the gastric polyp. Gene ontology analysis showed that upregulated genes were significantly enriched in the positive regulation of cell proliferation, epithelium development and angiogenesis. A protein-protein interaction analysis suggested that INHBA was at the center of the interaction network and might play an important role in CCS. Immunohistochemistry confirmed that INHBA expression was upregulated in CCS gastric polyps.

CCS is a rare disease and its diagnosis mainly depends on typical clinical manifestations, endoscopic findings and histological features. INHBA upregulation may contribute to its pathogenesis.