Disseminated bronchoalveolar keratinocyte plugs in severe neonatal congenital lamellar ichthyosis.

We present here a case of congenital lamellar ichthyosis responsible for neonatal death immediately after delivery due to obstruction by bonchoalveolar keratinocyte plugs. The main interest of this case was to confirm in utero amniotic fluid movement into the distal airways by fetal breathing or gasping.