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|Year : 2012 | Volume
| Issue : 4 | Page : 304-306
Coexistent primary eosinophilic enteritis and enteritis cystica profunda
R Chaturvedi, S Acharya, PA Gupte, AS Joshi
Department of Pathology, Seth GS Medical College and KEM Hospital, Mumbai, Maharashtra, India
|Date of Web Publication||4-Jan-2013|
Department of Pathology, Seth GS Medical College and KEM Hospital, Mumbai, Maharashtra
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Chaturvedi R, Acharya S, Gupte P A, Joshi A S. Coexistent primary eosinophilic enteritis and enteritis cystica profunda. J Postgrad Med 2012;58:304-6
Primary eosinophilic enteritis (EEn), one of the eosinophilic gastrointestinal disorders, is characterized by eosinophil-rich inflammation selectively involving the intestinal wall in the absence of any underlying cause for eosinophilia such as parasitic infection, drug reaction, or malignancy.  Enteritis cystica profunda (EnCP), which shows mucin-filled cysts lined by non-neoplastic columnar to flattened epithelium seen predominantly in the submucosa of the small intestine, though often seen by pathologists, has only about 21 reports in the literature. In this report, we present a case with a simultaneous occurrence of these two entities.
A 35-year-old female presented in the emergency department in February 2011 with complaints of pain in abdomen since 6 months and history of constipation and obstipation for 2 days. She had no history of fever, vomiting, diarrhea, exposure to herbal medication or drug reaction. The patient had no past or family history of allergy. There was no history suggesting tuberculosis or inflammatory bowel disease. There was no history of any major surgery in the past except tubal ligation done 8 years back. On examination there was diffuse tenderness found in her abdomen. Hematological investigations revealed a total leucocyte count of 12,000 per cu mm with 80% polymorphs and 20% lymphocytes. Her hemoglobin was 13.5 gm/dL and the platelet count was 2,50,000 per cu mm. Other investigations included blood urea nitrogen of 6.8 mg/dL, serum creatinine 0.6 mg/dL, random blood sugar 88 mg/dL, serum bilirubin 0.6 mg/dL, serum aspartate transaminase 24 IU/L, serum alanine transaminase 19 IU/L, serum amylase 126 IU/L, serum sodium 124 meq/L, serum potassium 3.2 meq/L and serum chloride 104 meq/L. Serological investigations for HIV 1, 2 (antibodies by ELISA) and Hepatitis B surface antigen (by ELISA) were negative. Contrast-enhanced CT scan of abdomen and pelvis demonstrated multiple fluid-filled dilated loops of small intestine suggesting stricture.
On exploratory laparotomy, an ileal stricture was seen approximately three feet proximal to the ileo-caecal junction. The proximal small bowel was edematous and dilated. Omental adhesions were seen at the site of previous tubal ligation. A double barrel ileostomy was performed after resection of the stricture.
A 7.5 cm long ileal segment was received for histopathological examination. External examination showed congested serosa and two strictures, each measuring 1.5 cm in length, at a distance of 1.5 cm and 4.0 cm from the distal resection margin. There was no evidence of tubercles or perforation. The bowel wall was 1cm thick at the stricture site and the lumen was 0.5 cm in diameter. The mucosa over one of the strictures showed a transverse circumferential ulcer measuring 1 cm × 3 cm.
Microscopy of the stricture revealed focal mucosal ulceration and mixed inflammation comprising of lymphocytes, plasma cells, neutrophils, and eosinophils. The submucosa showed multiple cystically dilated glands lined by low columnar, cuboidal to flat epithelium, a few of which contained scant mucinous material [Figure 1]. A dense inflammatory infiltrate predominantly composed of eosinophils (>20/high power field and about 100/high power field in occasional areas) was seen in the muscularis propria causing splaying of the muscle fibers, focally reaching the serosal surface [Figure 2], with prominent interstitial edema. Multiple sections studied did not show any parasite, granuloma, vasculitis, or mucosal dysplasia. Adjacent bowel showed mild mucosal inflammation with collections of eosinophils in the muscularis propria, predominantly involving the inner circular layer. These features were consistent with the diagnosis of primary eosinophilic enteritis with enteritis cystica profunda.
|Figure 1: Cystically dilated glands in the submucosa. (a) H and E, ×40 (b) H and E, ×100|
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|Figure 2: Dense eosinophilic infiltrate in the muscularis propria (a,b) and subserosa (c,d). (a) H and E, ×100 (b) H and E, ×400 (c) H and E, ×100 (d) H and E, ×400|
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Eosinophilic gastrointestinal disorders include eosinophilic esophagitis, eosinophilic gastritis, eosinophilic enteritis, and eosinophilic colitis, which can be primary or secondary. Based on the site of involvement, primary EEn has been classified into three subtypes, namely mucosal, muscular (or mural), and subserosal (or serosal) forms.  One or more of these layers can be affected and the symptoms vary accordingly. Predominant mucosal involvement causes diarrhea, subserosal involvement causes ascites, while muscular involvement can lead to thickening and induration of the intestinal wall resulting in obstruction and abdominal pain as seen in our patient. Abnormal eosinophilic infiltration of the intestine is defined by the presence at least 20 eosinophils per high power field which can be either diffuse or multifocal.  This criterion was fulfilled in our case. Peripheral eosinophilia, seen in about 80% of the patients,  is not a prerequisite for the diagnosis of EEn and was absent in our case. Though stool examination is an important tool to rule out parasitic infection which is an important cause of peripheral or tissue eosinophilia, this investigation could not be performed in our patient as she directly presented in the emergency department. However, histological examination did not show any evidence of parasite. EEn is associated with asthma and allergies in 40% to 50% of cases. In our patient, clinical features and history did not suggest any allergic disorder, and also there was absence of peripheral eosinophilia. As there were no pointers toward allergic disorder, specific investigations for the same were not done.
The pathogenesis of EnCP is believed to be similar to that of the commoner entity colitis cystic profunda. Although very little information is available regarding the exact etiology of EnCP, it is largely considered to be an acquired condition usually seen in association with diseases that tend to irritate the bowel due to mucosal ulceration or inflammatory damage to the submucosa.  It has been reported in association with Crohn's disease, Peutz-Jeghers syndrome More Details, Ileoileal intussusception, and trauma to the bowel.  Although EnCP with tuberculosis has not been reported, it might be a common association in our country. None of the known associated lesions were found in our case and EEn could have led to EnCP by causing mucosal ulceration and inflammation.
EnCP in association with EEn has not been reported so far in the literature. To the best of our knowledge, this is the first such case reported.
| :: Acknowledgment|| |
Dr. Pravin H. Shinde, Assistant Professor, Department of General Surgery, Seth GS Medical College and KEM Hospital, Mumbai, India.
| :: References|| |
|1.||Lim KC, Tan HK, Rajnakova A, Venkatesh SK. Eosinophilic gastroenteritis presenting with duodenal obstruction and ascites. Ann Acad Med Singapore 2011;40:379-81. |
|2.||Ng CF, Hull DA, Feakins RM, Baithun S, Dorudi S. Enteritis Cystica Profunda. J R Soc Med 2004;97:29-30. |
|3.||Chao JC, Lucha PA Jr. Enteritis cystica profunda: Is trauma the etiology? Interval development in the previously normal ileum: A case report and literature review. Mil Med 2008;173:513-4. |
[Figure 1], [Figure 2]
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