Oral Retinoid Treatment in a Newborn With Lamellar Ichthyosis

Ichthyosis is a heterogeneous group of conification disorders characterized by hyperkeratosis involving the skin. One form of ichthyosis, lamellar ichthyosis (LI), is autosomal recessively inherited with an incidence of 1/300,000. The diagnosis is based on clinical findings, skin biopsy and genetic analysis. Treatment includes moisturizing the skin, reducing fluid loss and keratolytics. Oral retinoids have been found to be very successful in the treatment of LI(1). In this case, a premature newborn diagnosed with LI is presented because of its rarity and good response to oral retinoid treatment.