Ichthyosis, Lamellar

Important

It is possible that the main title of the report Ichthyosis, Lamellar is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

Synonyms

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• Collodion Baby
• Congenital Ichthyosiform Erythroderma
• Desquamation of Newborn
• Lamellar Ichthyosis
• Non-bullous Congenital Ichthyosiform Erythroderma

Disorder Subdivisions

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• None

General Discussion

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Lamellar ichthyosis (LI or LI-1) is a rare skin disorder inherited as a recessive trait. It is characterized by generalized, abnormally red (erythroderma), dry, rough, thick skin that may mask the redness of the erythroderma. Newborns may be covered with a transparent, membrane (collodion) that has the appearance of a plastic wrap and that will be sloughed off, leaving large plate-like scales. Upturned eyelids (ectropion) expose their delicate linings, making them vulnerable to infection.



Clinical geneticists recognize at least five forms of lamellar ichthyosis. However, forms other than LI-1 are limited to a very few families, primarily located in countries bordering the Mediterranean.

Resources

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Foundation for Ichthyosis & Related Skin Types

1601 Valley Forge Road

Lansdale, PA 19446

Tel: (215)631-1411

Fax: (215)631-1413

Tel: (800)545-3286

Email: info@scalyskin.org

Internet: http://www.scalyskin.org



National Eczema Association for Science and Education

6600 SW 92nd Ave.

Suite 300

Portland, OR 97223-7195

Tel: (503)244-7404

Fax: (503)245-0626

Tel: (800)723-9166

Email: getinfo@psoriasis.org

Internet: http://www.psoriasis.org/home



National Registry for Ichthyosis and Related Disorders

University of Washington

Dermatology Department, Box 356524

1959 N.E. Pacific

Seattle, WA 98195-6524

Tel: (206)616-3179

Fax: (206)616-6793

Tel: (800)595-1265

Email: ichreg@u.washington.edu

Internet: http://www.skinregistry.org

For a Complete Report

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This is an abstract of a report from the National Organization for Rare Disorders, Inc. ® (NORD). A copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html

The information provided in this report is not intended for diagnostic purposes. It is provided for informational purposes only. NORD recommends that affected individuals seek the advice or counsel of their own personal physicians.

It is possible that the title of this topic is not the name you selected. Please check the Synonyms listing to find the alternate name(s) and Disorder Subdivision(s) covered by this report.

This disease entry is based upon medical information available through the date at the end of the topic. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder.

For additional information and assistance about rare disorders, please contact the National Organization for Rare Disorders at P.O. Box 1968, Danbury, CT 06813-1968; phone (203) 744-0100; web site www.rarediseases.org or email orphan@rarediseases.org

Last Updated:   4/15/2005

Copyright   1986, 1987, 1990, 1992, 1993, 1996, 1997, 2004 National Organization for Rare Disorders, Inc.