Spanish Version
Print-Friendly
Bookmarks
bookmarks-menu

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.

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 is not normal and 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
  • Loss of balance of minerals in the body (electrolyte imbalance)
  • Breathing problems
  • Body temperature that is not 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:

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

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.

Richard G, Ringpfeil F. Ichthyoses, erythrokeratodermas, and related disorders. In: Bolognia JL, Schaffer JV, Cerroni L, eds. Dermatology. 4th ed. Philadelphia, PA: Elsevier; 2018:chap 57.

BACK TO TOP Text only

  • 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

  • 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: 10/14/2018

Reviewed By: Michael Lehrer, MD, Clinical Associate Professor, Department of Dermatology, University of Pennsylvania Medical Center, Philadelphia, PA. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.

The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. © 1997- A.D.A.M., a business unit of Ebix, Inc. Any duplication or distribution of the information contained herein is strictly prohibited.
adam.com

 
 
 

 

 

A.D.A.M. content is best viewed in IE9 or above, Firefox and Google Chrome browser.
Content is best viewed in IE9 or above, Firefox and Google Chrome browser.