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Unfortunately, there is no cure for lamellar ichthyosis. Management is generally symptomatic and supportive. For neonates, providing a moist environment in an isolette, preventing infection by hygienic handling, and treating infection are most important. Petrolatum-based creams and ointments are used to keep the skin soft, supple, and hydrated. As affected children become older, keratolytic agents such as alpha-hydroxy acid or urea preparations may be used to promote peeling and thinning of the stratum corneum (the outermost layer of skin cells). For individuals with ectropion (turning out of the eyelid), lubrication of the cornea with artificial tears or prescription ointments, especially at night, is helpful in preventing desiccation (drying out) of the cornea. Oral retinoid therapy may be recommended for those with severe skin involvement; however, side effects of this include bone toxicity and other complications. This type of therapy should be used with caution in women of child-bearing age because of concerns about teratogenicity (harm that the therapy may cause an unborn fetus).
Last updated: 6/23/2011
- Sherri J Bale, Gabriele Richard. Autosomal Recessive Congenital Ichthyosis. GeneReviews. November 19, 2009; http://www.ncbi.nlm.nih.gov/books/NBK1420/#li-ar.Management. Accessed 6/23/2011.
- 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.
- 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.
- 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.