We encountered a case of a 56-year-old woman with Cronkhite-Canada syndrome who presented
with chronic diarrhea, alopecia, intermittent abdominal pain, hyperpigmentation, and
nail dystrophy (Figure [1]). Her face, palms, and the back of her hands and soles of her feet were deeply pigmented
with small dark brown spots. Her family history was noncontributory. Serum total protein
was 5.4 g/dl, with 2.4 g/dl of albumin. Endoscopic examination of the stomach showed
multiple sessile polyps, varying in size from 2 mm to 10 mm throughout the entire
stomach and duodenum (Figure [2]). Small-bowel x-ray and colonoscopic examination revealed multiple polyps from the
jejunum to the rectum. Histopathologic examination of these polyps revealed edema
of the lamina propria, and mucosal erosion associated with evidence of chronic inflammation.
Scintigraphy with technetium-99m-labeled human albumin demonstrated a protein-losing
enteropathy. Hyperalimentation (2200kcal/d) was continued for 8 weeks. At 1 week later,
the patient’s diarrhea showed a decrease in severity, with improvement in appetite
and weight gain. However, the diarrhea recurred after 2 weeks. After administration
of prednisolone, 30 mg daily, and trimethoprim with sulfamethoxazole (Bactrim), clinical
improvement was noted, with cessation of diarrhea, increased serum protein, disappearance
of pigmentation, and regrowth of the scalp hair.
Figure 1The patient’s nails showed dystrophy such as splitting and partial separation from
the nail bed
Figure 2Endoscopic view of the stomach, showing multiple sessile polyps, varying from 2 mm
to 10 mm in size
Cronkhite-Canada syndrome has a poor prognosis because of malnutrition resulting from
altered absorption in the gastrointestinal tract [1]. The diarrhea and hypoproteinemia seem to arise as a result of protein loss into
the gastrointestinal lumen. Many cases have had a fatal outcome [1]
[2]
, with a reported 6-month survival rate of 40 %, but some cases of spontaneous remission
have also been reported [3]. A partial recovery following administration of prednisolone suggests that it may
be effective in preventing a leakage of plasma protein into the gastrointestinal tract.
The accumulating evidence for remission in Cronkhite-Canada syndrome supports suggestions
that it might have nutritional, infective, toxic, or other exogenous causes [1]
[2]
. Malnutrition and metabolic disturbances may be responsible for the observed ectodermal
changes [4]. Although the possibility of a purely coincidental association between enteral nutrition
and recovery cannot be excluded, the sequence of events suggests that nutritional
support should be tried early in the course of this illness.
