Clinical Snapshot
Sclerosing Cholangitis Owing to Langerhans Cell Histiocytosis
;
A 63-year-old man had an 8-week history of fever, weight loss, and fatigue. Laboratory tests showed elevated parameters of cholestasis. Endoscopic retrograde cholangiography shows sclerosing cholangitis (Figure). Biopsy samples from the bile duct revealed a histiocytic infiltrate with CD1a- and S100-positive cells. Only rarely does sclerosing cholangitis occur due to Langerhans cell histiocytosis.
Langerhans cell histiocytosis is a rare disease that infiltrates one or more organs. It is caused by the clonal expansion of myeloid cells that differentiate to CD1a/CD207+ cells. The result is a broad spectrum of organ involvement (skin, skeleton, lymph nodes, lungs, liver, spleen, bone marrow, central nervous system). In our patient the bile ducts are affected, an extremely rare form in adulthood with less than 20 cases described. No BRAF mutation was detected. The patient’s symptoms resolved spontaneously, and no systemic organ involvement was found. For this reason no antiproliferative treatment has yet been initiated. We have performed endoscopic dilatation of the bile ducts.
Dr. med. Klaus Muehlenberg, Prof. Dr. med. Oliver Pech, Krankenhaus Barmherzige Brüder Regensburg, Klinik für Gastroenterologie und interventionelle Endoskopie, klaus.muehlenberg@barmherzige-regensburg. de
Conflict of interest statement: The authors declare that no conflict of interest exists.
Translated from the original German by David Roseveare.
Cite this as: Muehlenberg K, Pech O: Sclerosing cholangitis owing to Langerhans cell histiocytosis. Dtsch Arztebl Int 2022; 119: 686. DOI: 10.3238/arztebl.m2022.0123
