Congenital Langerhans Cell Histiocytosis with Placental Involvement

Abstract

Congenital presentations of Langerhans cell histiocytosis (LCH) are exceedingly rare, and concurrent placental parenchymal involvement has not been definitively documented in the literature. We present 2 cases of congenital multisystem LCH with placental involvement resembling chronic villitis. Placental examination may provide the initial diagnostic evidence of LCH and may significantly influence patient management and outcome; however, the prognostic implications remain unclear. In a clinical context suspicious for congenital LCH, the observation of chronic villitis should prompt consideration of placental involvement by LCH.