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Genetic and Rare Diseases Information Center (GARD)

Lamellar ichthyosis

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Lamellar ichthyosis is a rare genetic skin disorder in which the skin cells are produced at a normal rate, but they do not separate normally at the surface of the skin and are not shed as quickly as they should be; this results in the formation of scales. Lamellar ichthyosis is present at birth; many babies born with the condition are covered with a clear membrane (the collodion) and have skin that can be red or dark, tight and split. The eyelids and lips may be forced open by the tightness of the skin (which may continue into adulthood), and there may be contractures around the fingers. Other signs and symptoms may include problems with temperature regulation, water loss, secondary infections, thickened nails, and hair loss. The condition may be caused by mutations in any of several different genes and is usually inherited in an autosomal recessive manner. Treatment may include moisturizers, keratolytics, and oral synthetic retinoids (in severe cases).[1]
Last updated: 6/23/2011


  1. Ichthyosis, Lamellar. NORD. March 31, 208; Accessed 6/23/2011.
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Basic Information

  • DermNet NZ is an online resource about skin diseases developed by the New Zealand Dermatological Society Incorporated. DermNet NZ provides information about this condition.
  • Genetics Home Reference (GHR) contains information on Lamellar ichthyosis. This website is maintained by the National Library of Medicine.
  • MedlinePlus was designed by the National Library of Medicine to help you research your health questions, and it provides more information about this topic.
  • The National Organization for Rare Disorders (NORD) is a federation of more than 130 nonprofit voluntary health organizations serving people with rare disorders. Click on the link to view information on this topic.

In Depth Information

  • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
  • Online Mendelian Inheritance in Man (OMIM) is a catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine. 
    Lamellar ichthyosis 1
    Lamellar ichthyosis 2
    Lamellar ichthyosis 3
    Lamellar ichthyosis, autosomal dominant
    Lamellar ichthyosis 5
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs.  Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Lamellar ichthyosis. Click on the link to view a sample search on this topic.
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.