Chediak-Higashi syndrome with intestinal complication. Report of a case.

A 21-year-old woman with Chediak-Higashi syndrome developed intestinal lesions similar to those of Crohn's disease. Radiographic findings showed a stenotic lesion with multiple nodular changes in the ascending colon and cecum, and a longitudinal ulceration in the terminal ileum. In addition, multiple oval-shaped ulcerations were seen between the terminal ileum and descending colon. However, serial histological examinations revealed only an infiltration of lymphoid cells, which was a nonspecific finding. Though there are few reports of gastrointestinal involvement in Chediak-Higashi syndrome, such an intestinal complication may develop in patients long surviving with this disease.