Chronic Gastroenteritis in a Patient with Histiocytosis-X

We describe a 5-year-old white girl who presented at 18 months of age with an ileal perforation and radiographic evidence of segmental intestinal narrowing. Her initial diagnosis was Crohn's disease; however, on subsequent microscopic review, her lesion consisted of mixed histiocytic and eosinophilic infiltration characteristic of histiocytosis-X. The case is unique in its clinical similarity to Crohn's disease and its paucity of extraintestinal manifestations in the face of persistent mild chronic intestinal symptoms. She remains well controlled on prednisone therapy.