Lamellar ichthyosis

Lamellar ichthyosis (LI) is a rare skin condition. It appears at birth and continues throughout life.

Causes

LI is an autosomal recessive disease. This means that the mother and father must both pass one abnormal copy of the disease gene to their child in order for the child to develop the disease.

Autosomal recessive disease

Autosomal recessive is one of several ways that a trait, disorder, or disease can be passed down through families. An autosomal recessive disorder me...

Image

Read Article Now Book Mark Article

Symptoms

Many babies with LI are born with a clear, shiny, waxy layer of skin called a collodion membrane. For this reason, these babies are known as collodion babies. The membrane sheds within the first 2 weeks of life. The skin underneath the membrane is red and scaly resembling the surface of a fish.

With LI, the outer layer of skin called the epidermis cannot protect the body like the healthy epidermis can. As a result, a baby with LI may have the following health problems:

• Difficulty in feeding
• Loss of fluid (dehydration)

  Dehydration

  Dehydration occurs when your body does not have as much water and fluids as it needs. Dehydration can be mild, moderate, or severe, based on how much...

  

  Image

  Read Article Now Book Mark Article
• Loss of balance of minerals in the body (electrolyte imbalance)

  Electrolyte

  Electrolytes are minerals in your blood and other body fluids that carry an electric charge. Electrolytes affect how your body functions in many ways...

  Read Article Now Book Mark Article
• Breathing problems
• Body temperature that is not stable
• Skin or body-wide infections

Older children and adults with LI may have these symptoms:

• Giant scales that cover most of the body
• Decreased ability to sweat, causing sensitivity to heat
• Hair loss
• Abnormal finger and toenails
• Skin of the palms and soles is thickened

Treatment

Collodion babies usually need to stay in the neonatal intensive care unit (NICU). They are placed in a high-humidity incubator. They will need extra feedings. Moisturizers need to be applied to the skin. After the collodion membrane is shed, babies can usually go home.

Lifelong care of the skin involves keeping the skin moist to minimize the thickness of the scales. Measures include:

• Moisturizers applied to the skin
• Medicines called retinoids that are taken by mouth in severe cases
• High-humidity environment
• Bathing to loosen scales

Possible Complications

Babies are at risk for infection when they shed the collodion membrane.

Eye problems may occur later in life because the eyes cannot close completely.