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Toxic Epidermal Necrolysis

National Organization for Rare Disorders, Inc.

Important

It is possible that the main title of the report Toxic Epidermal Necrolysis 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

  • Acute Toxic Epidermolysis
  • Dermatitis Exfoliativa
  • Lyell Syndrome
  • Ritter Disease
  • Ritter-Lyell Syndrome
  • Scalded Skin Syndrome
  • Staphyloccal Scalded Skin Syndrome
  • TEN
  • Toxic Epidermal Necrolysis
  • Lyelles Syndrome

Disorder Subdivisions

  • None

General Discussion

Toxic epidermal necrolysis (TEN) is a rare, sometimes life-threatening unless properly treated, immunological disorder of the skin. It is characterized by blisters that meld into one another to cover a substantial portion of the body (30% and more), and extensive peeling or sloughing off of skin (exfoliation and denudation). The exposed under layer of skin (dermis) is red and suggests severe scalding. Often, the mucous membranes become involved, especially around the eyes (conjunctivitis), but also the mouth, throat, and bronchial tree.



Onset can occur at any age. The infantile form frequently follows an infection. In adults the disorder is usually caused by a reaction to taking a pharmaceutical drug, especially anticonvulsants, non-steroid anti-inflammatories, and/or some antibiotics.



TEN is thought to be an immunological disorder and to be one of a family of three skin disorders. TEN is considered to be the more serious, followed by Stevens-Johnson syndrome and erythema multiforme, in order of severity of disease.

Resources

Dystrophic Epidermolysis Bullosa Research Association of America, Inc. (DEBRA)

16 East 41st Street

3rd Floor

New York, NY 10017

Tel: (212)868-1573

Fax: (212)868-9296

Tel: (866)332-7276

Email: staff@debra.org

Internet: http://www.debra.org



NIH/National Institute of Arthritis and Musculoskeletal and Skin Diseases

Information Clearinghouse

One AMS Circle

Bethesda, MD 20892-3675

USA

Tel: (301)495-4484

Fax: (301)718-6366

Tel: (877)226-4267

TDD: (301)565-2966

Email: NIAMSinfo@mail.nih.gov

Internet: http://www.niams.nih.gov/



University of Pennsylvania Dermatology Clinic

Dermatology Clinic

34th and Spruce Streets

Philadelphia, PA 19104

Tel: (215)662-2737

Tel: (800)789-7366

Internet: http://www.uphs.upenn.edu/dermatol/



Stevens Johnson Syndrome Foundation and Support Group

PO Box 350333

Westminster, CO 80035-0333

Tel: (303)635-1241

Fax: (303)648-6686

Email: sjs@gmail.com

Internet: http://www.sjsupport.org



Genetic and Rare Diseases (GARD) Information Center

PO Box 8126

Gaithersburg, MD 20898-8126

Tel: (301)251-4925

Fax: (301)251-4911

Tel: (888)205-2311

TDD: (888)205-3223

Internet: http://rarediseases.info.nih.gov/GARD/



For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders (NORD). A copy of the complete report can be downloaded free from the NORD website for registered users. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational therapies (if available), and references from medical literature. For a full-text version of this topic, go to MyD-H, the Dartmouth-Hitchcock patient portal. You must be a registered MyD-H user for the Lebanon, Manchester, or Nashua locations to access this site.

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:  4/23/2008

Copyright  1987, 1989, 1996, 2004 National Organization for Rare Disorders, Inc.

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