First case of dizygotic twins with lamellar ichthyosis in Puerto Rico.

Lamellar Ichthyosis is a rare condition requiring thickened collodion-like skin for clinical diagnosis. These infants have abnormal epidermal barrier, which leads to serious complications. It may present with extensive skin lesions and should be considered a diagnostic possibility in sick neonates. Recent studies have identified a condition characterized by deficiency of the interleukin-1 receptor antagonist (DIRA) resembling Ichthyosis. We report dizygotic twins that presented lamellar ichthyosis. Twin #1 had a neonatal period complicated with sepsis, fungemia and urinary tract infection. Twin #2 had sepsis, urinary tract infection and conjunctivitis. We tested the parents and one patient for DIRA. Tests were negative. To our knowledge, this is the first case of dizygotic twins that presented with lamellar ichthyosis in Puerto Rico. Physicians should include DIRA in the differential diagnosis of skin lesions in newborns.