Çok düşük doğum ağırlıklı bir bebekte konjenital iktiyoz

The ichthyoses are clinically and etiologically heterogeneous group of hereditary keratinization disorders. They are characterized by generalized scaling and dryness of the skin, occasionally by erythroderma and histopathologically by hyperkeratosis. Ichthyosis vulgaris and X-linked ichthyosis are the most common types of ichthyosis. Congenital ichthyoses are rare, which include lamellar ichthyosis, congenital ichthyosiform erythroderma, and harlequin ichthyosis. Lameller ichthyoses are usually present at birth or present soon after birth.  The affected infants are born with a parchment paper like transparent membrane (collodion membrane). Prevention of the complications such as dehydration, sepsis, electrolyte imbalance and pneumonia is important for survival. The main goal of the treatment is to prevention of fluid loss, emollition of the skin and providing the softness of stratum corneum.  In our case very low birth weight premature infant with the diagnosis of lamellar ichthyosis based on clinically findings was treated successfully only with topical agents.  Hereby, we reviewed the care of premature infants with congenital ichthyosis in neonatal intensive care unit. Key words: very low birth weight, prematurity, neonatal intensive care unit, congenital ichthyosis