Rosai–Dorfman disease in the distal ileum causing intestinal obstruction

Abstract

Rosai–Dorfman disease (RDD) is an infrequent non-Langerhans cell histiocytosis characterised by the proliferation of histiocytes with emperipolesis. Although extranodal involvement accounts for 43% of cases, gastrointestinal involvement is uncommon (<1%), making isolated ileal disease extremely rare. We report the case of a 66-year-old male presenting with intestinal obstruction preceded by constitutional symptoms. Capsule endoscopy demonstrated an ulcerated, stenosing lesion in the distal ileum, resulting in capsule retention and requiring surgical intervention. Histopathological examination confirmed RDD and excluded IgG4-related disease. This case illustrates the diagnostic challenge of extranodal RDD, which must be considered as a differential diagnosis in gastrointestinal lesions with atypical behaviour, to ensure timely management and prevent complications associated with delayed diagnosis.