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Ichthyosis congenita with biliary atresia: a rare association.

Clinical summary Ichthyosis refers to a group of inherited skin disorders characterized by abnormal epidermal scaling. Congenital ichthyosis is known to be associated with various extracutaneous defects grouped as syndromic ichthyosis. However, the association of ichthyosis with biliary atresia is extremely rare and has been reported only in one family in the medical literature back in 1854 (Gould, 1854).