Gottron Syndrome

National Organization for Rare Disorders, Inc.

Important
It is possible that the main title of the report Gottron Syndrome 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

  • familial acrogeria
  • familial acromicria
  • H. Gottron's syndrome
  • acrogeria, Gottron type

Disorder Subdivisions

  • None

General Discussion

Gottron syndrome (GS) is an extremely rare inherited disorder characterized by the appearance of premature aging (progeria), especially in the form of unusually fragile, thin skin on the hands and feet (distal extremities). GS is described as a mild, nonprogressive, congenital form of skin atrophy due to the loss of the fatty tissue directly under the skin (subcutaneous atrophy). Other findings may include abnormally small hands and feet with unusually prominent veins on the chest; small stature; and/or abnormally small jaw (micrognathia).

Other characteristics that develop later in life may include premature senility, endocrine disturbances and cataracts. Gottron syndrome is thought to be inherited as an autosomal recessive genetic trait. Only about 40 cases have been reported in the medical literature.

There is some debate in the literature regarding a possible relationship between Gottron syndrome and Ehlers-Danlos syndrome, type IV. Some clinicians believe the terms are synonymous. Others disagree.

Resources

March of Dimes Birth Defects Foundation
1275 Mamaroneck Avenue
White Plains, NY 10605
Tel: (914)997-4488
Fax: (914)997-4763
Tel: (888)663-4637
Email: Askus@marchofdimes.com
Internet: http://www.marchofdimes.com

Progeria Research Foundation, Inc.
2 Bourbon Street
Suite 208
Peabody, MA 01960
USA
Tel: (978)535-2594
Fax: (978)535-5849
Email: info@progeriaresearch.org
Internet: http://www.progeriaresearch.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 www.rarediseases.org and click on Rare Disease Database under "Rare Disease Information".

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:  3/30/2008
Copyright  1986, 1988, 1994, 1997, 2005 National Organization for Rare Disorders, Inc.

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