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Infantile colitis: a manifestation of intestinal Behcet's syndrome
S K Chong1, V M Wright, T Nishigame
1King's College Hospital, London, England.
Insights
Pediatric inflammatory bowel disease can manifest as Behçet's syndrome, presenting with chronic diarrhea and mouth ulcers. This rare condition in children requires consideration in differential diagnoses, even after surgical intervention.
Area of Science:
- Pediatric Gastroenterology
- Immunology
- Genetics
Background:
- Recurrent mouth ulcers and chronic diarrhea in neonates suggest complex gastrointestinal issues.
- Standard treatments like prednisolone and sulphasalazine were ineffective, indicating a need for alternative diagnoses.
- The familial occurrence in siblings with Pakistani parental consanguinity suggests a potential genetic component.
Abstract:
A brother and sister of first cousin Pakistani parents presented with recurrent mouth ulcers and chronic diarrhoea in the neonatal period. Diarrhoea persisted in spite of treatment with oral prednisolone and sulphasalazine. Both children required subtotal colectomy with ileostomy. Histopathology of the resected colons was virtually identical and showed multiple deep "flask"-shaped ulcers, often penetrating to the serosa, in the presence of chronic inflammation, but without any of the characteristic histological features of Crohn's disease. The appearance closely resembled the colitis of Beh,cet's syndrome. Both children (aged 6 and 3.5 years, respectively) have continued to have recurrent perianal disease with intermittent bloody diarrhoea since the operation. Apart from the initial symptoms of oral aphthous ulcerations in both children, no other major criteria have developed so far. Intestinal Behçet's syndrome should be considered in the differential diagnosis of chronic inflammatory bowel disease in childhood.