Case Snippets

frequent location. Gastrointestinal and retroperitoneal sites were each identified in 12 percent of cases.1 There is a bimodal age incidence, with a small cluster of cases occurring in childhood.2 A female-to-male preponderance of 4:1 is reported. The cardinal histologic finding is the presence of numerous histiocytes with pathognomonic intracellular and extracellular inclusions known as Michaelis-Gutmann bodies, which typically stain positively with periodic acid-Schiff, von Kossa, and Prussian blue stains.
An association between immunosuppression and malakoplakia has been documented.3 Chronic granulomatous disease (CGD) is a primary immunedeficient
state manifested by defective phagocytosis.
4 Celiac disease is also believed to be due to immune dysfunction.5
A 4-year-old boy was hospitalized for chronic diarrhea,
severe malnutrition and abdominal pain. He was a normal 3300-gram neonate at birth with normal growth and development during the first year of life. He had been hospitalized one year ago for chronic diarrhea. At that time anti-endomysial antibody test was positive twice and duodenal biopsy showed moderate duodenitis with moderate
villous atrophy. He was on gluten-free diet, but because
of no response over 9 months, his parents discontinued
the regimen.
His brother had had chronic intractable diarrhea and recurrent skin infections with no response to gluten-free diet. He died due to an unknown infection at age 6 years.

On examination he was a cachectic child with 9.5 Kg weight, 85 cm height and 49 cm head circumference. He had pale conjunctiva, brittle hair, distended abdomen and no organomegaly. His mental status was normal.
Stool examination was negative for parasites. Immunologic
assessment was near normal (35% T cells, 28% B cells, 74% CD4, 17% CD8) except for positive nitro-bluetetrazolium
test. Anti-HIV and anti-HTLV1 were also negative.
On colonoscopy he had erythematous mucosa in the rectosigmoid and descending colon; biopsy showed Michaelis-Gutmann bodies.
After starting ciprofloxacin followed by cotrimoxacole,
on regular diet, his diarrhea stopped. His weight increased by 2.5 Kg after one month but bloating and abdominal distension did not resolve. We reassessed him for celiac disease. Anti-tissue transglutaminase test was positive. Duodenal biopsy showed partial villous atrophy;
cryptosporidium infestation was also seen.
To our knowledge, this is the first case reported
of gastrointestinal malakoplakia in a child, occurring with CGD and celiac disease.
The etiology of malakoplakia is unclear. Current evidence points to a defect in macrophage killing activity. Nondigested micro-organisms are found within the lysosomes of macrophages in affected persons. Macrophages from these patients show a decrease

found to have a single colonic polyp, which was removed; biopsy revealed adenomatous polyp with no dysplasia.
Classically the polyps in Peutz-Jegher syndrome are hamartomatous, but adenomas and adenocarcinomas
are also reported. Hizawa et al2 described his experience with 75 GI polyps from 7 patients with PJS where 71 had hamartomatous polyps, 2 adenoma, 1 cancer in adenoma, and 1 pyogenic granuloma.
Small bowel polyps are treated with laparotomy with enteroscopy and polypectomy. Our patient had developed colonic and ovarian malignancies along with small bowel polyps; over just a two-year follow
up, all of these were succesfully treated. A hamartoma-adenoma-carcinoma sequence as been described
in PJS.2
References
1. Burdick D, Prior JT. Peutz-Jeghers syndrome. A clinicopathologic
study of a large family with a 27-year followup.
Cancer 1982;50:2139-46.
2. Hizawa K, Iida M, Matsumoto T, Kohrogi N, Yao T, Fujishima M. Neoplastic transformation arising in Peutz-Jeghers polyposis. Dis Colon Rectum 1993;36:953-7.