Failure of etretinate therapy in twins with severe congenital lamellar ichthyosis

We cared for twin females with severe lamellar exfoliation of the newborn (collodion babies) and a family history of death of two brothers at age 3 months. The twins were given a controlled trial of etretinate (Ro 10-9359, Tigason) after not responding to other therapeutic modalities. One of the infants received 1 mg/kg body weight of the drug for 24 hours, while her identical twin sister did not have the medication. Monthly skin biopsies taken from both patients during nine months of therapy did not demonstrate any histologic difference under light microscopy. The clinical course in both infants was basically the same. Etretinate plasma level measurements proved that the drug was absorbed from the gastrointestinal tract. We conclude that in the very severe forms of congenital lamellar ichthyosis, oral retinoids may fail to produce any beneficial effects.