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

[lamellar ichthyosis]

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 .