Gottron Syndrome

Important
It is possible that the main title of the report Gottron Syndromeis not the name you expected.

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)428-7100
Fax: (914)997-4763
800: (888)663-4637
Askus@marchofdimes.com
http://www.marchofdimes.com

Progeria Research Foundation, Inc.
532 Lowell Street
Peabody
MA
01961-3453
USA
Tel: (978)535-2594
Fax: (978)535-5849
info@progeriaresearch.org
http://www.progeriaresearch.org

MUMS (Mothers United for Moral Support, Inc) National Parent-to-Parent Network
150 Custer Court
Green Bay
WI
54301-1243
USA
Tel: (920)336-5333
Fax: (920)339-0995
800: (877)336-5333
mums@netnet.net
http://www.netnet.net/mums/

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

WebMD Medical Reference from the National Organization of Rare Disorders

Last Updated: March 30, 2008

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