Cronkhite-Canada Syndrome Workup

  • Author: Agnieszka B Serwin, MD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Jan 24, 2012
 

Laboratory Studies

  • Electrolyte and micronutrient determination - Hypokalemia; hypocalcemia; depressed serum levels of zinc, iron, copper, and magnesium; and vitamin B-12
  • Hematology - Depressed white blood cell count, hemoglobin, red blood cell count, and hematocrit
  • Serum proteins - Hypoproteinemia with hypoalbuminemia; increased in alpha1 globulin level
  • Raised erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP)
  • Elevated serum levels of gastrin, histamine-fast achlorhydria, hypochlorhydria.
  • Biochemical and hematologic tests - Total protein level; albumin level; glucose and lipids concentration; iron, magnesium, zinc, calcium, sodium, potassium, and copper concentrations; ESR; CRP
  • Decreased cholinesterase activity
  • Stool examination - Occult blood and Sudan III staining
  • H pylori infection test
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Imaging Studies

  • Endoscopic (also wireless capsule endoscopy[23] ) procedures (ie, gastroscopy, colonoscopy, sigmoidoscopy) reveal polyp lesions of the sessile or semipedunculated type throughout the stomach, duodenum, ileum, and colon, sparing the esophagus.
    • Ikeda et al found pedunculated polyps in patients with Cronkhite-Canada syndrome. Their size varied from a few millimeters to 2 cm in diameter.[24]
    • Endoscopic findings in the stomach also include reddish and edematous granular lesions with mucoid exudate and giant folds.
    • Abdominal CT scanning may reveal thickened gastric folds.
  • Regarding radiography, a barium enema and small intestine double-contrast radiology examination show polypoid lesions.
  • A CT endoscopy using a multidetector-row CT scan with 3-dimensional reconstruction has been shown to be useful for the detection of Cronkhite-Canada syndrome polyps and for the monitoring of effects of therapy.[25]
  • Magnified chromoendoscopy with crystal violet reveals sparsely distributed crypt openings with widening of the pericryptal space on the surface of the polyps and the intervening colonic mucosa.[26]
  • Fluoroscopic examination of the stomach may show rough granular changes of the mucosa with edematous giant rugae and polypoid lesions.
  • Scintigraphy using technetium Tc 99m–labeled human albumin may result in leakage to the gastrointestinal tract.
  • Consider intraoperative or postoperative examination of p55 immunoreactivity accumulation in the tissue suspected of having a carcinomatous component.
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Other Tests

  • Presence of antinuclear antibodies, usually with a nucleolar pattern
  • Malignancy markers (carcinoembryonic antigen, alpha-fetoprotein)
  • D-xylose absorption test (impaired)
  • Glucose tolerance test (may be impaired)
  • Fecal fat excretion (increased)
  • Gastrointestinal clearance of alpha-1 antitrypsin (usually elevated)
  • Culture of nail scrapings for fungi (for differential diagnosis)
  • HIV testing (possibly)
  • Stool culture for Salmonella, Shigella, Yersinia, and Campylobacter species and for parasites.
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Procedures

  • Endoscopic procedures (gastroscopic, duodenoscopic, and colonoscopic examinations)
  • X-ray procedures (eg, barium enema examination, small intestine double-contrast radiology after Sellink)
  • Fluoroscopic examination of the gastrointestinal tract
  • Abdominal computed tomographic scan
  • Endoscopic biopsy of the polypoid lesion
  • Histologic examination of polypoid lesions and, if necessary, the scalp lesion
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Histologic Findings

Hyperpigmentation is related to an increase in melanin within the basal layer without the melanocyte proliferation.

Scalp biopsy shows a marked noninflammatory loss of follicular units, miniaturization of the hair shafts, markedly dilated follicles, and a heavy deposition of glycosaminoglycans in the reticular dermis.

Histologically, polyps in patients with Cronkhite-Canada syndrome are pseudopolypoid-inflammatory changes with cystic dilatation. Ikeda et al found that some of the colon polyps presented a histologic pattern of a tubulovillous adenoma and others exhibited that of a juvenile-type polyp.[24] The latter polyps were characterized by the presence of elongated and/or tortuous crypts with microcystic dilatation and inflamed edematous wide stroma. The areas of focal intestinal metaplasia were present. Even though cellular atypia was present, the adenomatous polyps showed histologic similarity to juvenile polyps with inflamed edematous stroma and occasional cystic glands.

According to Burke and Sobin,[27] Cronkhite-Canada syndrome polyps are characterized by their broad sessile base, expanded edematous lamina propria, and cystic glands. The only reliable distinction between Cronkhite-Canada syndrome and colonic juvenile polyposis is the pedunculated growth of the latter with the exception of the gastric polyps. Gastric polyps in Cronkhite-Canada syndrome are sessile and composed of focally dilated irregular foveolar glands within a lamina propria expanded by edema and often an inflammatory infiltrate. Most polyps contain smooth muscle fibers in the lamina propria, and a minority has surface erosions. Gastric Cronkhite-Canada syndrome polyps are quite similar to juvenile or hyperplastic polyps.

The most constant features of Cronkhite-Canada syndrome polyps are a sessile base, an expanded edematous lamina propria, and dilated glands. Other features, including inflammation, a small number of smooth muscle fibers, and a complex contour, are variable.

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Contributor Information and Disclosures
Author

Agnieszka B Serwin, MD  Consulting Staff, Department of Dermatology and Venereology, Medical Academy of Bialystok, Poland

Disclosure: Nothing to disclose.

Coauthor(s)

Hanna Mysliwiec, MD  Staff Physician, Department of Dermatology and Venereology, Medical Academy of Bialystok, Poland

Disclosure: Nothing to disclose.

Specialty Editor Board

Franklin Flowers, MD  Chief, Division of Dermatology, Professor, Department of Medicine and Otolaryngology, Affiliate Associate Professor of Pediatrics and Pathology, University of Florida College of Medicine

Franklin Flowers, MD, is a member of the following medical societies: American College of Mohs Micrographic Surgery and Cutaneous Oncology

Disclosure: Nothing to disclose.

David F Butler, MD  Professor of Dermatology, Texas A&M University College of Medicine; Chair, Department of Dermatology, Director, Dermatology Residency Training Program, Scott and White Clinic, Northside Clinic

David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Association of Military Dermatologists, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Robert A Schwartz, MD, MPH  Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, University of Medicine and Dentistry of New Jersey-New Jersey Medical School

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and Sigma Xi

Disclosure: Nothing to disclose.

Catherine M Quirk, MD  Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania

Catherine M Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD  Director, Ackerman Academy of Dermatopathology, New York

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

References

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  28. Allbritton J, Simmons-O'Brien E, Hutcheons D, Whitmore SE. Cronkhite-Canada syndrome: report of two cases, biopsy findings in the associated alopecia, and a new treatment option. Cutis. Apr 1998;61(4):229-32. [Medline].

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