Eosinophilic gastroenteritis, first reported by Kaijser in 1937,is a rare disorder that can present with various gastrointestinalmanifestations, depending on the site of the affectedgastrointestinal wall. Klein et al[1] have demonstrated that thisdisorder may be histopathologically classified into three majortypes: predominant mucosal, predominant muscle, andpredominant subserosal layers. However, its clinical features,aetiology, and treatment have not yet been definitely established.We report a rare case of eosinophilic gastroenteritis withfeatures of the predominant muscle layer type, with ascites,abdominal distension, diarrhoea and high grade fever.

Case Report

A 45-year-male patient presented with complaints of abdominaldistension, occasional vomiting, high grade intermittent feverand increased frequency of stool for one month. There was nosignificant history of any other illness. He had a normal appetiteand no allergy to any food item. There were no positive findingson general examination. On per abdominal examination, theabdomen was distended, soft, and non-tender, with no guardingor rigidity; the liver and spleen were not palpable, suggestingthe presence of intra-abdominal free fluid. On per rectalexamination fullness was appreciated. His complete bloodwas evaluated in which total counts rose to 15291/µL, absoluteeosinophilic counts were 8410/µL,  55% of the total WBC count.His urine analysis, renal functions and liver functions werenormal. The chest x-ray revealed right pleural effusion and x-rayabdomen in upright position showed ground glass appearancewith few air fluid bowel loops. The ultrasound abdomen andthorax suggested moderate ascites and thickened bowel loops.CT scan confirmed the ultrasound findings and additionallyshowed diffuse thickened mucosal folds in t entire small bowelloops. Diagnostic ascitic tap was carried out which reportedreddish yellow turbid fluid; the cell count was 8060 cells/µL inwhich the polymorphs were 90% and lymphocytes 10%. Smearswith gram and ZN stains were negative. Ascitic fluid culture wasnegative. Ascitic fluid adenosine deaminase test was negative.On cytological examination no malignant cells were seen. Heunderwent upper gastrointestinal endoscopy andsigmoidoscopy to rule out infiltrative disease and storagedisease which were not conclusive on histopathology. Then itwas decided to take full thickness biopsy from the bowel,peritoneum and from the liver through diagnostic laparoscopy.On laparoscopy there was moderate ascites with diffuselydilated small bowel. Biopsies were taken from liver, peritoneumand jejunum. On histopathological examination liver tissuewas normal, peritoneal biopsy showed infiltration of the tissueby chronic inflammatory cells along with eosinophils; jejunalbiopsy consisted chiefly of the muscularis propria which wasinfiltrated by eosinophils. Thus his diagnosis confirmedeosinophilic gastroenteritis. He was prescribed prednisolone20 mg once a day for 6 weeks which was then tapered off. Hisascites was relieved within 15 days, clinical improvementnoted within one month and he was followed up for one yearafter stopping the drug and did not show recurrence of symptoms.

Discussion

Eosinophilic gastroenteritis was first reported by Kaijser in1937. In 1970, Klein[1] classified the disease according to thepredominance of eosinophilic infiltration in the different layersof the intestinal wall. Talley et al[2] identified four main diagnosticcriteria: (1) the presence of gastrointestinal symptoms, (2)biopsies demonstrating eosinophilic infiltration in one or moreparts of the gastrointestinal tract, (3) absence of eosinophilicinvolvement of multiple organs outside the GI tract and (4) noevidence of parasitic or extra-intestinal disease. Disease israre, undiagnosed and surely underreported.[1,2]  Patients typicallypresent in the third through fifth decades of life, but the diseasecan affect any age group. An equal gender distribution or a slightmale preponderance has been reported.[2] The cause is unknownand pathogenesis is poorly understood. Any segment of the GItract may be involved, including the oesophagus[4,5] or colon,[6] but most commonly the stomach or small bowel is affected.Classification is based on the layer of the gut wall primarilyaffected. Most prevalent form is characterised by mucosal (andsubmucosal ) disease.[1,2,3] Predominant mucosal layer diseasepresents with pain, nausea, vomiting, diarrhoea, weight loss,iron deficiency anaemia, malabsorption, and protein loosingenteropathy, whilst predominantly muscle layer diseasepresents with the obstructive features. The rarest form isserosal disease; all layers of the bowel are usually involved,and patients present with typical ascites.[1,2,7] Only rarely ismucosal involvement absent where serosal disease ispresent[7]. However, the definitive diagnosis of eosinophilicgastroenteritis requires histological evidence of eosinophilicinfiltration. Blood investigation shows peripheral eosinophiliain about 80% cases; the absolute eosinophil count averages2000 cells/µL in patients with mucosal disease and 1000 cells/µL in patients with disease of the muscle layer, although thecount may fluctuate markedly over time.[2] Other findings includeiron deficiency anaemia, hypoalbuminemia. Radiologicalchanges found in eosinophilic gastroenteritis are variable,nonspecific, and absent in at least 40% of patients.[8,9] The gastric folds are enlarged, with or without nodular filling defects.CT may demonstrate thickened intestinal wall and localisedmesenteric lymphadenopathy; with serosal involvement; asciticfluid is usually detected. When subserosal disease involvesthe small bowel, biopsy of the mucosal layer taken duringgastroscopy or colonoscopy often fails to diagnose eosinophilicgastroenteritis. Laparotomy or laparoscopic full thicknessbiopsy of the bowel is often required for confirmation in suchcases. On histopathology it is characterised by oedema and aninflammatory cell infiltrate that is almost entirely composed ofeosinophils; the eosinophils may occur in clumps.[10] Treatment with steroids is the mainstay in the management of eosinophilicgastroenteritis. Other modalities include diet modification,anti-helminthic, and mast cell stabilisers (sodiumchromoglycate).  Surgical intervention is required only when adefinitive diagnosis cannot be made or when complicationslike obstruction, perforation or bleeding occur. Because thenatural history of eosinophilic gastroenteritis has not been welldocumented, long-term follow-up is required.

References

1. Klein NC, Hargrove RL, Sleisenger MH, Jeffries GH. Eosinophilicgastroenteritis. Medicine (Baltimore). 1970;49:299–319.
2. Talley NJ, Shorter RG, Phillips SF, Zinsmeister AR. Eosinophilicgastroenteritis: a clinicopathological study of patients with diseaseof the mucosa, muscle layer, and subserosal tissues. Gut.1990;31:54–8.
3. Kalantar SJ, Marks R, Lambert JR, Badov D, Talley NJ. Dyspepsiadue to eosinophilic gastroenteritis. Dig Dis Sci. 1997;42:2327–32.4.
4. Katz AJ, Goldman H, Grand RJ. Gastric mucosal biopsy in eosinophilic(allergic) gasteroenteritis. Gastroenterology. 1977;73:705–9.
5. Walsh SV, Antonioli DA, Goldman H, Fox VL, Bousvaros A,Leichtner AM, et al. Allergic esophagitis in children: aclinicopathological entity. Am J Surg Pathol. 1999;23:390–6.
6. Naylor AR, Pollet JE. Eosinophilic colitis. Dis Colon Rectum.1985;28:615–8.
7. McNabb PC, Fleming CR, Higgins JA, Davis GL. Transmural eosinophilicgastroenteritis with ascites. Mayo Clin Proc. 1979;54:119–22.
8. MacCarty RL, Talley NJ. Barium studies in diffuse eosinophilicgastroenteritis. Gastrointest Radiol. 1990;15:183–7.
9. Stallmeyer MJ, Crew FS. Eosinophilic gastroenteritis. AJR Am JRoentgenol. 1993;161:296.
10. Johnstone JM, Morson BC. Eosinophilic gastroenteritis.Histopathology. 1978;2:335–48.