Ulcerative colitis is a chronic inflammatory bowel disease characterized by recurrent bloody diarrhea in association with acute and chronic inflammation, usually limited to the colonic mucosa. Although the etiology of ulcerative colitis remains unknown, it is generally believed to be multifactorial. The disease occurs in all age groups, with variable incidence and prevalence in different countries.^1-4 In Saudi Arabia, there are a few reports of ulcerative colitis in adults,⁵ but to the best of our knowledge, the disease has not been previously reported in Saudi children. We describe a documented case of ulcerative colitis in childhood to emphasize the need to include this disease in the differential diagnosis of chronic bloody diarrhea in children.

Case

A 10-year-old Saudi girl presented with bloody diarrhea of three months' duration. There was no associated fever, vomiting, or abdominal pain but her appetite was poor. Initial evaluation in another hospital revealed sickle cell anemia and stool examination for ova and parasites and cultures for enteropathogens yielded negative findings. The patient was then referred to our facility for further investigation. There was no history of exposure to drugs, irradiation, or allergy to food. The patient is from Al-Hasa, Eastern Province, and has three brothers and three sisters. There was no history of intolerance to food or bowelproblems in the parents or siblings. However, one of the sisters has sickle cell anemia.

Physical examination revealed a mildly ill-looking patient with pallor of the mucous membranes. Vital signs, including temperature, were normal. Her weight was 20 kg and height, 1.11 meters, both below the third percentile. Remaining physical examination findings were negative; in particular, there was no jaundice, skin rash, joint involvement, hepatosplenomegaly, abdominal distention, or perianal disease.

Laboratory studies showed white blood cells, 14.3 × 10⁹/L; polymorphs, 0.62; lymphocytes, 0.29; eosinophils, 0.01; hemoglobin, 81 g/L; reticulocytes, 0.123; platelets, 480 × 10⁹/L; and normal red blood cell indices. Purified protein derivative testing was negative; erythrocyte sedimentation rate was 38 mm/h, and hemoglobin electrophoresis showed S of 0.78, F of 0.20, and A₂ of 0.02. Total serum proteins and albumin determinations, liver function tests, renal function tests, and urinalysis were within normal limits. Humoral and cell-mediated immunologic studies were not available. Stool samples were positive for occult blood and there were 10 to 15 polymorphs per high power field.

Microscopy examination of stool and biopsy material and cultures were negative for ova, parasites, enteropathogenic bacteria, and mycobacteria on three different occasions. Barium enema showed loss of haustration with multiple spiculations involving the entire colon (Figure 1). Limited colonoscopy revealed red, friable mucosa with petechiae, erosions, and ulcerations, more severe in the rectosigmoid than in the descending colon, in a continuous pattern, but no plaques or polyps (Figure 2). Histopathologic examination revealed intact mucosa. The lamina propria was expanded by an inflammatory infiltrate rich in his tiocytes and eosinophils, with no significant goblet cell depletion, cryptitis, granuloma, Michaelis-Gutmann bodies, or signs of ischemic colitis.

At this stage, the diagnosis of chronic, nonspecific colitis was made. After two months of treatment with sulfasalazine, the patient's bloody diarrhea stopped and her hemoglobin stabilized without blood transfusion for eight months. However, her appetite was still decreased, weight gainwas unsatisfactory, and stools continued to show occult blood.

Colonoscopy was repeated 14 months after onset of illness and showed marked improvement, but histopathologic examination revealed increased expansion of the lamina propria. In addition to histiocytes and eosinophils, there were also lymphocytes and neutrophils. Focal areas of acute cryptitis were seen but no definite crypt abscesses. There was significant loss of goblet cells and distortion of the glands with branching and budding (Figure 3). Focal collagenosis and hyperplasia of the mucosal lymphoid tissue with definite germinal centers were present in other sections of the biopsy, but there were no granulomas or signs of ischemia or malacoplakia.

Discussion

The clinical presentation and the radiologic and endoscopic features in our patient are consistent with the diagnosis of ulcerative colitis. Although the initial histopathologic features were those of nonspecific colitis, the histopathology of the colonic biopsy material after 14 months of illness was more characteristic of ulcerative colitis. The difficulty in making an early diagnosis of ulcerative colitis, when other causes of chronic colitis are still possible, is well recognized. In our patient, the diagnosis was made only after a long period of observation and repeated investigations that ruledout other causes of chronic colitis. Initially it was considered that the sickle cell anemia could be the cause of colitis, probably on the basis of bowel ischemia. However, ischemic colitis usually presents suddenly, abdominal pain predominates, the rectal ampulla is usually preserved, and the histopathologic findings consist of mucosal necrosis and ballooning of capillaries with red cell congestion.⁶ Such features were not present in our patient. In addition, in view of the high incidence and mild features of sickle cell anemia in eastern Saudi children, the association of ulcerative colitis and sickle cell anemia in our patient appears coincidental.⁷

Other causes of chronic colitis, such as allergic colitis, pseudomembranous colitis, and hemolytic-uremic syndrome, are unlikely on the basis of the clinical and laboratory findings. However, the differential diagnosis with colonic malacoplakia deserves special attention. This chronic disease has been reported in Saudi children more frequently than in any other population.^8,9 The clinical presentation and radiologic and sometimes endoscopic features are similar to those of ulcerative colitis, but the histopathologic features leading to a diagnosis of malacoplakia are the presence of histiocytic infiltrate and Michaelis-Gutmann bodies in the colonic mucosa. In our patient there were no histopathologic signs of malacoplakia.

In view of the difference in treatment, colonic malacoplakia and ulcerative colitis should be distinguished from each other. The apparent lack of previous reports of ulcerative colitis in Saudi children may reflect a low index of suspicion, limited diagnostic facilities, or low incidence of presentation in children.

References

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2. Myren J, Gjone E, Hertzberg JN, et al. Epidemiology of ulcerative colitis and regional enterocolitis in Norway. Scand J Gastroenterol 1971;6:511-4.

3. Das SK, Montgomery RD. Chronic inflammatory bowel disease in Asian immigrants. Practitioner 1978;221:747-9.

4. Al-Nakib B, Radhakrishnan S, Jacob GS, et al. Inflammatory bowel disease in Kuwait. Am J Gastroenterol 1984;79:191-4.

5. Al-Freihi HM, Al-Idrissi HY, Al-Quorain A, et al. The pattern of colonic diseases in the Eastern Province of Saudi Arabia. J Trop Med Hyg 1986;89:23-7.

6. Roth JLA. Diagnosis and differential diagnosis of chronic ulcerative colitis and Crohn's disease. In: Kirsner JB, Shorter RG, eds. Inflammatory bowel disease. Philadelphia: Lea & Febiger, 1980:166-202.

7. Al-Awamy B, El-Mouzan MI, Al-Torki, et al. Neonatal screening for sickle cell in the Eastern Province of Saudi Arabia. Trans R Soc Trop Med Hyg 1984;78:792-4.

8. Harfi HA, Akhtar MN, Subayti YA, et al. Gastrointestinal malacoplakia in children. Clin Pediatr 1985;24:423-8.

9. El Mouzan MI, Satti MB, Al-Quorain A, El-Ageb A. Colonic malacoplakia: occurrence in a family. Dis Colon Rectum 1988;31:390-3.