Successful treatment of Cronkhite–Canadasyndrome using anti-tumor necrosis factorantibody therapy

Cronkhite–Canada syndrome (CCS) is arare nonhereditary syndrome character-ized by gastrointestinal polyposis andectodermal changes [1]. Although severaltreatments, such as steroids, are available,the prognosis is poor, with a 5-year mor-tality rate of 55% [2].A 57-year-old man, who suffered fromalopecia, anorexia, severe diarrhea, dys-trophic nail changes, and pigmentationon the hands, presented to our hospital.

Colonoscopy revealed numerous polypsthroughout the colon (●" Fig.1a). Gastro-scopy and small-bowel series also showedsevere gastric and small-bowel polyposis,and histology showed juvenile-like polypswith mild inflammation. On the basis ofthese findings, the patient was diagnosedwith CCS.He was treated with medications includ-ing corticosteroids, antiplasmin agents,and azathioprine; however, the symp-toms persisted. He developed intussus-ception 7 years after diagnosis (●" Fig.1b)and underwent ileocecal resection. As hecontinued to suffer from frequent relap-ses, with diarrhea and malnutrition withsevere hypoalbuminemia, a different ther-apeutic strategy was needed to controlthe disease progression. Supported by asingle report finding of high levels oftumor necrosis factor alpha (TNF-α) in tis-sue affected by CCS [3], implying that anti-TNF-α therapy could be useful for CCSpatients, the patient was administeredinfliximab at 200mg every 2 weeks. Hisgeneral condition improved, and colonos-copy performed 20 months after induc-tion of anti-TNF therapy showed completeremission with the disappearance ofpolyposis (●" Fig.1c). He now receives200mg of infliximab every 8 weeks, andhas been symptom free for 3 years sincethe initial administration.There is one report that described strongTNF-α expression in intestinal mucosaaffected by CCS, implicating the potentialusefulness of anti-TNF-α antibody for pa-tients with CCS. However, to the best of

our knowledge, this treatment has notbeen tested until now [3]. Although wecould not detect TNF-α expression in thepolyps of this patient by immunohisto-chemistry (●" Fig.2), given the patient’sdramatic response to therapy, it can be as-sumed that TNF plays an important role indisease development, perhaps upstreamof polyp formation. This case indicatesthat further basic and clinical research iswarranted, and that anti-TNF therapycould be an important new strategy fortreatment of CCS.

Endoscopy_UCTN_Code_CCL_1AD_2AC

Competing interests: None

Daisuke Watanabe1, Makoto Ooi1,Namiko Hoshi1, Michitaka Kohashi1,Tomoo Yoshie1, Nobunao Ikehara1,Masaru Yoshida2, Emmy Yanagita3,Takashi Yamasaki3, Tomoo Itoh3,Takeshi Azuma1

1 Division of Gastroenterology, Depart-ment of Internal Medicine, Kobe Univer-sity Graduate School of Medicine, Kobe,Japan

2 Division of Metabolomics Research,Department of Internal Medicine, KobeUniversity Graduate School of Medicine,Kobe, Japan

3 Division of Diagnostic Pathology,Department of Pathology, Kobe Univer-sity Graduate School of Medicine, Kobe,Japan

Fig.1 Colonoscopy in a patient with Cronkhite–Canada syndrome. a Before initial treatment. b 3 days before ileocecal resection. c 20months after anti-tumornecrosis factor alpha therapy.

Fig.2 Immunohistochemistry for tumor necro-sis factor alpha (TNF-α). Colon tissue of patientwith Cronkhite–Canada syndrome was stainedwith: a anti-TNF-α antibody; b isotype control.

Cases and Techniques Library (CTL)E476

Watanabe Daisuke et al. Treatment of Cronkhite–Canada syndrome using anti-TNF antibody therapy… Endoscopy 2014; 46: E476–E477

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References1 Cronkhite LW Jr, Canada WJ. Generalized

gastrointestinal polyposis; an unusual syn-drome of polyposis, pigmentation, alopeciaand onychotrophia. N Engl J Med 1955;252: 1011–1015

2 Nakayama M, Muta H, Somada S et al. Cron-khite–Canada syndrome associated withschizophrenia. Intern Med 2007; 46: 175–180

3 Martinek J, Chvatalova T, Zavada F et al. Afulminant course of Cronkhite–Canada syn-drome. Endoscopy 2010; 42: E350–351

BibliographyDOI http://dx.doi.org/10.1055/s-0034-1377539Endoscopy 2014; 46: E476–E477© Georg Thieme Verlag KGStuttgart · New YorkISSN 0013-726X

Corresponding authorTakeshi Azuma, MD, PhDDivision of GastroenterologyDepartment of Internal MedicineKobe University7-5-1, Kusunoki-choChuo-ku, Kobe 650-0017JapanFax: +81-78-3826309azumat@med.kobe-u.ac.jp

Watanabe Daisuke et al. Treatment of Cronkhite–Canada syndrome using anti-TNF antibody therapy… Endoscopy 2014; 46: E476–E477

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