Gastrointestinal (GI) tract involvement by LCH is rare and may appear as a solitary colorectal polyp or multiple granulomatous lesions of the mucous membrane in the upper and lower GI tract [Singhi/Montgomery 2011]. Patients are often asymptomatic. Multiple infiltrations are associated with abdominal pain, diarrhea, and hypoalbuinemia.
Liver infiltration is characterized sometimes by infiltration of CD1a+ cells in nodules or by lymphocytes alone along the portal tracts which may lead to sclerosing cholangitis. In case of splenomegaly other causes than LCH primarily have to be ruled out. Pancreatic involvement (mainly tumorous) is extremely rare.