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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Nonbullous congenital ichthyosiform erythroderma


Other Names for this Disease
  • CIE
  • Congenital ichthyosiform erythroderma
  • Ichthyosiform erythroderma, Brocq congenital, nonbullous form
  • Ichthyosiform erythroderma, congenital, nonbullous, 1
  • NBCIE
More Names
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Treatment


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How might nonbullous congenital ichthyosiform erythroderma be treated?

There is currently no cure for nonbullous congenital ichthyosiform erythroderma (NBCIE). Treatment generally focuses on managing the specific signs and symptoms each individual has. For newborns, the most important goals are to provide a moist environment in an isolette, and to prevent and treat infections. Petrolatum-based creams and ointments are typically used to keep the skin soft and hydrated. As children age, keratolytic agents (agents that help the skin loosen and shed) such as alpha-hydroxy acid or urea preparations can be used to promote the peeling and thinning of the outer layer of the skin. For individuals with severe skin involvement, oral retinoid therapy may be recommended. However, because this is known to cause abnormalities in a developing fetus, it should be used with caution in women of child-bearing age. In general, any agents that irritate the skin should be avoided. Ectropion (turning out of the eyelid) can cause dryness of the cornea (especially at night), so artificial tears or prescription ointments may be used to keep the cornea moist.[1]

ClinicalTrials.gov provides access to information on clinical studies (including therapies) for different types of ichthyosis. To view a list of the studies currently listed, click here.
Last updated: 10/7/2011

References
  1. Sherri J Bale, Gabriele Richard. Autosomal Recessive Congenital Ichthyosis. GeneReviews. November 19, 2009; http://www.ncbi.nlm.nih.gov/books/NBK1420/. Accessed 10/7/2011.


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. Click on the link to view the article on this topic.

Clinical Trials & Research for this Disease

  • ClinicalTrials.gov lists trials that are studying or have studied Nonbullous congenital ichthyosiform erythroderma. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.