A 68-year-old white man presented with a 7-month history of anorexia, hypogeusia, and watery diarrhea, with an estimated weight loss of 40 lb during this period. Physical examination showed diffuse hyperpigmentation, alopecia, and onychodystrophy of the nails of the hands and feet. Laboratory evaluation revealed normochromic, normocytic anemia and decreased serum levels of ionized calcium (4.4 mg/dL; normal, 4.7 to 5.4 mg/dL), albumin (3.0 g/dL; normal, 3.5 to 5.0 g/dL), copper (0.73 µg/mL; normal, 0.75 to 1.45 µg/mL), ionized magnesium (0.51 mmol/L; normal, 0.53 to 0.67 mmol/L), zinc (0.61 µg/mL; normal, 0.66 to 1.1 µg/mL), iron (222 µg/dL; normal, 50 to 150 µg/dL), carotene (31 µg/dL; normal, 48 to 200 µg/dL), and retinol (256 µg/L, normal 360 to 1,200 µg/L). Radiologic studies including computed tomography (CT) of the abdomen were unremarkable. Upper and lower endoscopy revealed innumerable diminutive polyps in the stomach, duodenum, terminal ileum, and right colon. On histopathology, the lesions were consistent with hamartomatous polyps with myxoid expansion of the lamina propria, numerous degranulated eosinophils, and occasional mast cells. These characteristic endoscopic and pathologic findings as well as the classic ectodermal changes led to the diagnosis of CCS.
The patient was first treated with nutritional hyperalimentation with multivitamin and zinc supplementation. He initially responded with improvement in symptoms; however, within 6 months after beginning this therapy, he again began to lose weight. His course was also complicated by recurrent gram-negative bacterial sepsis and idiopathic thrombocytopenic purpura requiring splenectomy. At this point, oral combination therapy was started with prednisone (40 mg daily), cromolyn sodium (200 mg four times a day), ranitidine (150 mg twice a day), and loratadine (10 mg daily). This regimen was based on the possible role of mast cell dysfunction in the pathogenesis of this syndrome. Long-term suppressive antibiotic therapy was also given in the form of a fluoroquinolone (ciprofloxacin). Over the next 12 months, the patient's condition improved significantly, with marked regression of the intestinal polyposis, resolution of the diarrhea and hypoalbuminemia, weight gain, regrowth of body hair, and normalization of the nail and skin findings. Prednisone was then tapered and discontinued while treatment with the other medications (ranitidine, loratadine, and cromolyn) was continued.
At about this time, more than 2 years after the initial diagnosis, follow-up studies detected an abnormality in the pancreatic bed in association with a rising serum gastrin level (940 pg/mL; normal, 0 to 200 pg/mL) and elevated chromogranin A level (94.0 ng/mL; normal, 2.3 to 14.3 ng/mL). A CT scan of the abdomen revealed an area of abnormal signal attenuation in the head of the pancreas, but this finding was not seen on magnetic resonance cholangiopancreatography. Clinically, the patient began to have recurrent symptoms of diarrhea and weight loss. Ultimately, an octreotide scan and endoscopic ultrasound studies were done but failed to detect significant pancreaticoduodenal abnormalities. The corticosteroids were then restarted (prednisone 20 mg/day), and the patient's symptoms have promptly abated with reversal of weight loss and resolution of laboratory abnormalities, indicating disease remission.
South Med J. 2002;95(2) © 2002 Lippincott Williams & Wilkins
Cite this: Medical Management of Cronkhite-Canada Syndrome - Medscape - Feb 01, 2002.