Publication:

Solitary Langerhans cell histiocytosis of liver with sclerosing cholangitis in an adult female

Liver is affected as a late complication of high risk cases of Langerhans cell histiocytosis. Sclerosing cholangitis is a rare pattern associated with Langerhans cell histiocytosis of liver, which is even rarer in the adult population and has high mortality. The treatment is difficult and may require liver transplantation. We report a unique case of a 40-year-old female who developed sclerosing cholangitis associated with Langerhans cell histiocytosis without any evidence of involvement of other systems. Our patient required only surgery, and had been followed up for two years without recurrence. We could not find any other case of solitary liver involvement of Langerhans cell histiocytosis in literature published so far.