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Journal of Pediatric Gastroenterology & Nutrition:
doi: 10.1097/MPG.0b013e31820ea688
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Nodularity of the Small Intestine in a Child With Systemic Mastocytosis Associated With Hyperimmunoglobulin M Syndrome

Homan, Matjaž*; Avčin, Tadej

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*Department of Gastroenterology, Hepatology, and Nutrition

Department of Allergy, Rheumatology, and Clinical Immunology, University Children's Hospital, Ljubljana, Slovenia.

A 7-year-old boy with hyperimmunoglobulin M syndrome presented with intermittent bloody diarrhea since the age of 6 months. On physical examination he had hepatosplenomegaly, generalized lympadenopathy, and hyperpigmented papular/vesicular rash around the umbilicus. The growth parameters were normal. The skin biopsy was consistent with mastocytoma. Endoscopic evaluation revealed nodular small intestinal mucosa. The most prominent nodularity was seen in the duodenum (Fig. 1), and less so in the terminal ileum. The histology revealed acute and chronic inflammation without architectural changes, and a high number of mastocytes, confirming systemic mastocytosis (Fig. 2). Treatment with monthly intravenous gamma globulin, antihistamines, ketotifen, and proton pump inhibitor resulted in almost complete resolution of gastrointestinal symptoms.

Figure 1
Figure 1
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Figure 2
Figure 2
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Mastocytosis is characterized by excessive proliferation of mastocytes in several organs, most frequently skin, bones, lymph nodes, liver, spleen, and the gastrointestinal tract (1). Systemic mastocytosis is rare in children (2). Therapy is based on symptomatic treatment and avoiding triggers of mast cell mediator release. Long-term prognosis for pediatric-onset disease is better in contrast to adult-onset mastocytosis (3). Hyperimmunoglobulin M also can be associated with chronic diarrhea. Both Cryptosporidium and Giardia rarely cause nodularity of the small intestine (4); however, no etiologic microorganism was identified on stool testing. Therefore, given the histologic picture a showing high number of mastocytes, we presume that the symptoms and the nodular mucosal changes were caused by systemic mastocytosis.

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1. Katsamba AD, Karpouzis AJ, Koumantaki-Mathioudaki E, et al. Mastocytosis with skin manifestations: current status. J Eur Acad Dermatol Venereol 1999; 13:155–165.

2. Valent P, Horny HP, Escribano L, et al. Diagnostic criteria and classification of mastocytosis: a consensus proposal. Leuk Res 2001; 25:603–625.

3. Uzzaman A, Maric I, Noel P, et al. Pediatric-onset mastocytosis: a long term clinical follow-up and correlation with bone marrow histopathology. Pediatr Blood Cancer 2009; 53:629–634.

4. Pérez-Roldán F, Mate-Valdezate A, Villafáñez-García MC, et al. Nodular lymphoid hyperplasia by Giardia lamblia. Endoscopy 2008; 40 (suppl 2):E116–E117.

Copyright 2011 by ESPGHAN and NASPGHAN


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