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Case report of patient with a Cronkhite-Canada syndrome: sustained remission after treatment with corticosteroids and mesalazine.
BMC Gastroenterol. 2019 Feb 27; 19(1):36.BG

Abstract

BACKGROUND

Cronkhite-Canada syndrome is a rare disease of unknown etiology and the optimal treatment for this syndrome is unknown.

CASE PRESENTATION

We present the case of a man who at the age of 66.0 years was diagnosed with Cronkhite-Canada syndrome (CCS). In addition to watery diarrhea, alopecia, and a complete loss of toenails and fingernails, the patient had been suffering from dysgeusia and rapid weight loss of more than 10.0 kg within a few months. The patient had recently incurred a distal radius fracture. During the initial endoscopy an extensive polyposis of the stomach and jejunum was found. The diagnosis of CCS was made and after initiation of a steroid therapy his diarrhea improved immediately. A discontinuation of the steroid therapy was not possible and mesalazine (1000 mg t.i.d.) was added to prednisolone (10.0 mg/d). This therapy led to a remission within 6.0 months with weight gain and normalization of serum albumin levels. The prednisolone dose was reduced to 7.5 mg/d. During the following year, the steroids could be further reduced and nails had regrown again. Within three years, all polyps had disappeared and the steroid therapy was finished while the dosage of mesalazine was reduced in a stepwise fashion. Four years later, the mesalazine was stopped and more than 14.0 years after the initial diagnosis the patient is still in complete remission without any treatment.

CONCLUSION

The optimal treatment for CCS is unknown. In our case, the initial combination therapy of corticosteroids plus mesalazine followed by a mesalazine monotherapy has led to a remarkable long-lasting remission with complete resolution of all intestinal polyps.

Authors+Show Affiliations

Department of Gastroenterology and Hepatology, University Hospital of Cologne, D-50937, Cologne, Germany. sigrid.schulte@uk-koeln.de.Department of Gastroenterology and Hepatology, University Hospital of Cologne, D-50937, Cologne, Germany.Department of Gastroenterology and Hepatology, University Hospital of Cologne, D-50937, Cologne, Germany.Marienhospital, Mühlenstrasse 21, D-50321, Brühl, Germany.Institute of Pathology, University Hospital of Cologne, D-50937, Cologne, Germany.Department of Gastroenterology and Hepatology, University Hospital of Cologne, D-50937, Cologne, Germany.Department of Gastroenterology and Hepatology, University Hospital of Cologne, D-50937, Cologne, Germany.Department of Gastroenterology and Hepatology, University Hospital of Cologne, D-50937, Cologne, Germany.

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

30813906

Citation

Schulte, Sigrid, et al. "Case Report of Patient With a Cronkhite-Canada Syndrome: Sustained Remission After Treatment With Corticosteroids and Mesalazine." BMC Gastroenterology, vol. 19, no. 1, 2019, p. 36.
Schulte S, Kütting F, Mertens J, et al. Case report of patient with a Cronkhite-Canada syndrome: sustained remission after treatment with corticosteroids and mesalazine. BMC Gastroenterol. 2019;19(1):36.
Schulte, S., Kütting, F., Mertens, J., Kaufmann, T., Drebber, U., Nierhoff, D., Töx, U., & Steffen, H. M. (2019). Case report of patient with a Cronkhite-Canada syndrome: sustained remission after treatment with corticosteroids and mesalazine. BMC Gastroenterology, 19(1), 36. https://doi.org/10.1186/s12876-019-0944-x
Schulte S, et al. Case Report of Patient With a Cronkhite-Canada Syndrome: Sustained Remission After Treatment With Corticosteroids and Mesalazine. BMC Gastroenterol. 2019 Feb 27;19(1):36. PubMed PMID: 30813906.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Case report of patient with a Cronkhite-Canada syndrome: sustained remission after treatment with corticosteroids and mesalazine. AU - Schulte,Sigrid, AU - Kütting,Fabian, AU - Mertens,Jessica, AU - Kaufmann,Thomas, AU - Drebber,Uta, AU - Nierhoff,Dirk, AU - Töx,Ulrich, AU - Steffen,Hans-Michael, Y1 - 2019/02/27/ PY - 2018/10/15/received PY - 2019/01/28/accepted PY - 2019/3/1/entrez PY - 2019/3/1/pubmed PY - 2019/3/6/medline KW - Alopecia KW - Cronkhite-Canada syndrome KW - Mesalazine KW - Non-hereditary polyposis KW - Onychodystrophy SP - 36 EP - 36 JF - BMC gastroenterology JO - BMC Gastroenterol VL - 19 IS - 1 N2 - BACKGROUND: Cronkhite-Canada syndrome is a rare disease of unknown etiology and the optimal treatment for this syndrome is unknown. CASE PRESENTATION: We present the case of a man who at the age of 66.0 years was diagnosed with Cronkhite-Canada syndrome (CCS). In addition to watery diarrhea, alopecia, and a complete loss of toenails and fingernails, the patient had been suffering from dysgeusia and rapid weight loss of more than 10.0 kg within a few months. The patient had recently incurred a distal radius fracture. During the initial endoscopy an extensive polyposis of the stomach and jejunum was found. The diagnosis of CCS was made and after initiation of a steroid therapy his diarrhea improved immediately. A discontinuation of the steroid therapy was not possible and mesalazine (1000 mg t.i.d.) was added to prednisolone (10.0 mg/d). This therapy led to a remission within 6.0 months with weight gain and normalization of serum albumin levels. The prednisolone dose was reduced to 7.5 mg/d. During the following year, the steroids could be further reduced and nails had regrown again. Within three years, all polyps had disappeared and the steroid therapy was finished while the dosage of mesalazine was reduced in a stepwise fashion. Four years later, the mesalazine was stopped and more than 14.0 years after the initial diagnosis the patient is still in complete remission without any treatment. CONCLUSION: The optimal treatment for CCS is unknown. In our case, the initial combination therapy of corticosteroids plus mesalazine followed by a mesalazine monotherapy has led to a remarkable long-lasting remission with complete resolution of all intestinal polyps. SN - 1471-230X UR - https://www.unboundmedicine.com/medline/citation/30813906/Case_report_of_patient_with_a_Cronkhite_Canada_syndrome:_sustained_remission_after_treatment_with_corticosteroids_and_mesalazine_ L2 - https://bmcgastroenterol.biomedcentral.com/articles/10.1186/s12876-019-0944-x DB - PRIME DP - Unbound Medicine ER -