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

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

Disorder Subdivisions

None

General Discussion

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

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

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.

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