While Langerhans' cell histiocytosis exhibits the proliferation of CD1a(+), Langerin(+), S100(+) Langerhans' dendritic cells, ECD is hypothesized to originate from the proliferation and migration of CD68(+), CD1a(−) non Langerhans' histiocytes of monocyte-macrophage descent. Here, CD68 is linked to familial atrioventricular septal defect.