Severe congenital systemic juvenile xanthogranuloma with neonatal acute liver failure, neonatal hemochromatosis and placental infiltration

Juvenile xanthogranuloma(JXG) is a rare histiocytic disorder primarily affecting infants with self-limiting skin lesions. We present a severe case of a newborn who exhibited life-threatening multiorgan failure with systemic JXG involving the skin, bone marrow, liver and spleen. Notably, the placenta demonstrated infiltration by CD68+FXIIIa+ histiocytic cells and features of hemochromatosis. MRI of the patient revealed a novel finding of hepatic and pancreatic hemochromatosis in JXG. Our patient was salvaged with cytarabine and steroid-based chemotherapy and intensive care support. This case highlights the critical need for recognition of systemic JXG in neonates presenting with liver failure for timely intervention.