What is lamellar ichthyosis?

Lamellar ichthyosis, also called ichthyosis lammellaris or non -non -inborn ichthyosis, is a congenital skin condition characterized by hyperkeratosis or stratum corneum, the highest layer of skin. The result is a scaly appearance of the skin, especially on the neck, weakness, armpits, inner locations and similar joints. Lamellar ichthyosis is very rare and affects only one in 600,000 people. It is a mild form of ichthyosis, a group of genetic disorders that ranges from a very mild and relatively common vulgaris ichthyosis to life -threatening harlequin ichthyosis.

Children born with lamellar ichthyosis are referred to as "coldicial children" because they are born with a colodion membrane that looks like another layer of skin and shed it about two weeks after birth. The skin is shiny, waxy and tight and can pull out the eyes and mouth wide open. Due to their unusual skin, colodies are at risk of hypothermia, dehydration, leather infection and intoxication from topically applied products. Nursing in newbornThe intensive care unit until the colodinum membrane is not, often including the use of local humidifiers and a humidified incubator, helps to solve and prevent these risks. The condition is not painful or unpleasant for the child, although its complications can be.

6 about 15% have a different genetic skin disorder. Laboratory examination of the skin, blood or hair of the child can help doctors diagnose.

In addition to scales, thickened skin, lamellar ichthyosis in children and adults, it may cause overheating due to defective sweating, dry and irritated eyelids and temporary hair loss due to scaling on the scalp. Children with condition sometimes developing tight stripes around the fingers or fingers that reduce circulation. There is no cure for lamellar ichthyosis, but it can be relieved of humidifiers for skin and eye drops for dry eyes. In addition, patients should avoid hot weather and energetic exercise to makeprevented overheating.