$Unique_ID{BRK03782}
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$Title{Gottron's Syndrome}
$Subject{Gottron's Syndrome Familial Acrogeria Familial Acromicria}
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Copyright (C) 1986, 1988 National Organization for Rare Disorders,
Inc.

128:
Gottron's Syndrome

** IMPORTANT **
It is possible that the main title of the article (Gottron's Syndrome) is
not the name you expected.  Please check the SYNONYM listing to find the
alternate names and disorder subdivisions covered by this article.

Synonyms

     Familial Acrogeria
     Familial Acromicria

General Discussion

** REMINDER **
The information contained in the Rare Disease Database is provided for
educational purposes only.  It should not be used for diagnostic or treatment
purposes.  If you wish to obtain more information about this disorder, please
contact your personal physician and/or the agencies listed in the "Resources"
section.


Gottron's syndrome is a mild form of progeria, or premature ageing, in
which the extremities remain unusually small.  The disorder is familial.  The
prognosis for a normal life is good.

Symptoms

The hands and feet of patients with Gottron's syndrome remain small into
adulthood, with thin, parchment-like skin, so they seem much older than the
chronological age.  There is little subcutaneous fat in neither the
extremities nor on the chest so the veins on the chest are prominent.

Causes

Gottron's syndrome appears to be familial, but the inheritance pattern is not
understood.

Affected Population

Gottron's Syndrome affects males and females equally.

Related Disorders

Hutchinson-Gilford syndrome is a more severe form of progeria (premature
ageing) affecting children.  Werner syndrome, another form of progeria,
affects adults.  (For more information on these disorders, choose progeria,
Werner, and Hutchinson-Gilford as your search terms in the Rare Disease
Database.)

Therapies:  Standard

Treatment for Gottron's Syndrome is symptomatic and supportive.

Therapies:  Investigational

This disease entry is based upon medical information available through March
1987.  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.

Resources

For more information on Gottron's Syndrome, please contact:

     National Organization for Rare Disorders (NORD)
     P.O. Box 8923
     New Fairfield, CT  06812-1783
     (203) 746-6518

     The Progeria International Registry (PIR)
     New York State Institute for Basic Research in Developmental Disabilities
     1050 Forest Hill Road
     Staten Island, NY  10314
     (718) 494-0600

     Progeria Foundation
     3 Styvesant Oval, 9A
     New York, NY  10009

     Sunshine Foundation
     4010 Levick St.
     Philadelphia, PA  19135
     (The Sunshine Foundation raises funds to bring all children with Progeria
     together each year so that medical researchers can study their progress while
     the children socialize in a vacation atmosphere.)

     NIH/National Institute of Child Health and Human Development
     9000 Rockville Pike
     Bethesda, MD  20892
     (301) 496-5133

For information on genetics and genetic counseling referrals, please
contact:

     March of Dimes Birth Defects Foundation
     1275 Mamaroneck Avenue
     White Plains, NY  10605
     (914) 428-7100

     Alliance of Genetic Support Groups
     35 Wisconsin Circle, Suite 440
     Chevy Chase, MD  20815
     (800) 336-GENE
     (301) 652-5553

References

CECIL TEXTBOOK OF MEDICINE, 18th ed.:  James B. Wyngaarden, and Lloyd H.
Smith, Jr., Eds.:  W. B. Saunders Co., 1988.  P. 2035.