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202 CHRONIC COLLAGENOUS COLITIS IN A FOUR-YEAR-OLD GIRL: CASE REPORT AND REVIEW OF THE PEDIATRIC LITERATURE EI Benchimol, R Kirsch, S Viero, AM Griffiths INTRODUCTION: We present the first case of collagenous colitis (CC) associated with eosinophilic disease of the stomach, clinically responsive to mast cell stabilization therapy.
HISTORY: Currently 4½ years of age, this girl presented at age 15 months with acute onset of vomiting and watery diarrhea. She remained well but small, with weight and height at the 5th percentile for age, but growing appropriately. Investigations have included normal duodenal mucosal disaccharidases, hemoglobin, eosinophils, albumin, negative celiac serology, and positive pANCA (1/80). Esophogastroduodenoscopy and colonoscopy on two occasions have revealed similar findings but with histologic changes becoming more pronounced over time. The esophagus, stomach and duodenum were macroscopically normal. Histologically, gastric mucosa showed a prominent eosinophil infiltrate in the lamina propria with plasma cells and lymphocytes. The duodenum showed a mild increase in intraepithelial lymphocytes but was otherwise unremarkable. Endoscopically, the colon appeared edematous with no ulceration. Colonic biopsies all showed mild chronic inflammation in the superficial lamina propria and an increased numbers of intraepithelial lymphocytes. Sigmoid biopsy showed a thickened subepithelial collagen band, in areas exceeding 20 mm in width (normal 5-7 mm). Sulfasalazine therapy was not associated with clinical response. Oral ketotifen 0.2 mg/kg/day was associated with increased consistency and decreased frequency of stool.
DISCUSSION: CC has been previously described in only 9 other children, associated with a variety of conditions. Not previously associated with eosinophilic intestinal disease, increased eosinophilic cationic protein has been found in the sigmoid and rectum, and major basic protein in the colon of adults with CC. Eosinophils have found to produce TGF-beta1 in adults with CC, which is associated with production of tenascin and collagen type VI. TGF-beta1 has been associated with collagen deposition in hepatic fibrosis, systemic sclerosis and bleomycin-induced pulmonary fibrosis. Eosinophilic lysates produce fibroblast proliferation which may result in the collagenous band. Diarrhea may result from eosinophilic stimulation of the production of Substance P and vasoactive intestinal peptide.