label: autosomal recessive congenital ichthyosis 4B
label_lc: autosomal recessive congenital ichthyosis 4b
description: "Harlequin ichthyosis (HI) is the most severe variant of autosomal recessive congenital ichthyosis (ARCI). It is characterized at birth by the presence of large, thick, plate-like scales over the whole body associated with severe ectropion, eclabium, and flattened ears, that later develops into a severe scaling erythroderma."