Rosai–Dorfman disease with exclusive intra-abdominal lymphadenopathy masquerading as Wilkie’s syndrome

Abstract Rosai–Dorfman disease is a rare histiocytic proliferative disorder with a distinctive microscopic appearance. Patients usually present with nonspecific symptoms and massive cervical lymphadenopathy. Exclusive involvement of intra-abdominal lymph nodes is unusual and presentation mimicking Wilkie’s syndrome due to compression of the third part of the duodenum by enlarged retroduodenal lymph nodes is rare. This entity should be included in the differential diagnosis with infectious, granulomatous and malignant causes of intra-abdominal lymphadenopathy. We highlight an uncommon presentation and discuss the challenges in the diagnosis and management of Rosai–Dorfman disease.