Langerhans cell histiocytosis (LCH) is a rare disorder characterized by the clonal proliferation of Langerhans (CD1a/CD 207) cells. In adults, LCH is uncommon and typically presents as a multisystem disease affecting the lungs, bones, and pituitary gland. Primary hepatic involvement is exceedingly rare. We report the case of a 36-year-old man who presented with cholestatic jaundice. Subsequent lymph node and liver biopsies confirmed the diagnosis of multisystem LCH. The patient was initiated on prednisolone and demonstrated a favorable initial response. However, he ultimately succumbed to respiratory complications. This case highlights an atypical presentation of LCH where hepatic involvement preceded pulmonary symptoms. Early recognition is critical, as hepatic injury in the initial stages of LCH may be reversible.
Rajeev et al. (2025) studied this question.