November 30, 20250 citations

Hepatic Involvement as the Initial Manifestation of Adult Langerhans Cell Histiocytosis.

MRManu Rajeev ANAnanthu Narayan NANeha Agrawal

Key Points

Cholestatic jaundice marked the initial presentation of Langerhans cell histiocytosis in a 36-year-old man, leading to further investigation.
Biopsies confirmed multisystem disease characterized by Langerhans cell proliferation, emphasizing the rarity of this presentation.
The patient received prednisolone and showed a favorable response initially, though respiratory complications ultimately led to his death.
This case highlights the importance of early detection, as hepatic injury seen in initial stages may be reversible with prompt treatment.

Abstract

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.

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