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Lamellar ichthyosis
LI; Collodian baby - lamellar ichthyosis; Ichthyosis congenital; Autosomal recessive congenital ichthyosis - lamellar ichthyosis type
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...
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 collodian babies. The membrane sheds within the first 2 weeks of life. The skin underneath the membrane is red and scaly. It resembles the surface of a fish.
With LI, the outer layer of skin called the epidermis isn’t normal and can’t protect the body like the healthy epidermis can. As a result, a baby with LI may have the following health problems:
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Loss of fluid
dehydration
Dehydration
Dehydration occurs when your body does not have as much water and fluids as it should. Dehydration can be mild, moderate, or severe, based on how muc...
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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 isn't stable
- Skin or body-wide infections
Older children and adults with LI may have these symptoms:
- Very large 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
Support Groups
These groups can provide more information about LI:
- Foundation for Ichthyosis and Related Skin Types -- www.firstskinfoundation.org
- National Organization for Rare Disorders -- rarediseases.org/rare-diseases/ichthyosis-lamellar/
Possible Complications
Babies are at risk for infection when they shed the collodian membrane.
Eye problems may occur later in life because the eyes cannot close completely.
References
Hoath SB, Narendran V. The skin of the neonate. In: Martin RJ, Fanaroff AA, Walsh MC, eds. Fanaroff and Martin's Neonatal-Perinatal Medicine: Diseases of the Fetus and Infant . 10th ed. Philadelphia, PA: Elsevier Saunders; 2015:chap 102.
Martin KL. Disorders of keratinization. In: Kliegman RM, Stanton BF, St. Geme JW, Schor NF, eds. Nelson Textbook of Pediatrics . 20th ed. Philadelphia, PA: Elsevier; 2016:chap 658.
Paller AS, Mancini AJ. Hereditary disorders of cornification. In: Paller AS, Mancini AJ, eds. Hurwitz Clinical Pediatric Dermatology . 5th ed. Philadelphia, PA: Elsevier; 2016:chap 5.
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Ichthyosis, acquired - legs - illustration
Ichthyosis results from an abnormality in the cornification (production of the outer dead layer) of the skin. Many different forms of ichthyosis are recognized. Notice the dry skin and scaly appearance. Ichthyosis is usually worse in the winter and is more severe over the legs.
Ichthyosis, acquired - legs
illustration
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Ichthyosis, acquired - legs - illustration
Ichthyosis results from an abnormality in the cornification (production of the outer dead layer) of the skin. Many different forms of ichthyosis are recognized. Notice the dry skin and scaly appearance. Ichthyosis is usually worse in the winter and is more severe over the legs.
Ichthyosis, acquired - legs
illustration
Review Date: 10/24/2016
Reviewed By: David L. Swanson, MD, Vice Chair of Medical Dermatology, Associate Professor of Dermatology, Mayo Medical School, Scottsdale, AZ. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.
