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Diseases

Genetic and Rare Diseases Information Center (GARD)

Lamellar ichthyosis


Other Names for this Disease
  • Congenital lamellar ichthyosis
  • LI
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Treatment

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How might lamellar ichthyosis be treated?

Unfortunately, there is currently no cure for lamellar ichthyosis. Management is generally supportive and based on the signs and symptoms present in each person. For infants, providing a moist environment in an isolette (incubator) and preventing infection are most important. Petrolatum-based creams and ointments are used to keep the skin soft, supple, and hydrated. As affected children become older, treatments to promote peeling and thinning of the stratum corneum (the outermost layer of skin cells) are often recommended. This may include humidification with long baths, lubrication, and keratolytic agents such as alpha-hydroxy acid or urea preparations.[1]

For people with ectropion (turning out of the eyelid), lubrication of the cornea with artificial tears or prescription ointments is helpful to prevent the cornea from drying out. Topical or oral retinoid therapy may be recommended for those with severe skin involvement; however, these medications can be associated with undesired side effects and are, therefore, generally prescribed with caution.[1]
Last updated: 2/3/2016

References
  1. Gabriele Richard, MD, FACMG and Sherri J Bale, PhD, FACMG. Autosomal Recessive Congenital Ichthyosis. GeneReviews. August 2014; http://www.ncbi.nlm.nih.gov/books/NBK1420/.


Management Guidelines

  • GeneReviews provides current, expert-authored, peer-reviewed, full-text articles describing the application of genetic testing to the diagnosis, management, and genetic counseling of patients with specific inherited conditions.
  • Project OrphanAnesthesia is a project whose aim is to create peer-reviewed, readily accessible guidelines for patients with rare diseases and for the anesthesiologists caring for them. The project is a collaborative effort of the German Society of Anesthesiology and Intensive Care, Orphanet, the European Society of Pediatric Anesthesia, anesthetists and rare disease experts with the aim to contribute to patient safety.

Clinical Trials & Research for this Disease

  • ClinicalTrials.gov lists trials that are studying or have studied Lamellar ichthyosis. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.
Other Names for this Disease
  • Congenital lamellar ichthyosis
  • LI
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.