$Unique_ID{BRK04276}
$Pretitle{}
$Title{Tooth and Nail Syndrome}
$Subject{Tooth and Nail Syndrome Dysplasia of Nails With Hypodontia
Rapp-Hodgkin's Syndrome Nail Dystrophy-Deafness Syndrome Hidrotic Ectodermal
Dysplasias Anhidrotic Ectodermal Dysplasias }
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Copyright (C) 1988, 1989 National Organization for Rare Disorders, Inc.

537:
Tooth and Nail Syndrome

** IMPORTANT **
It is possible that the main title of the article (Tooth and Nail
Syndrome) is not the name you expected.  Please check the synonym list to
find the alternate names and disorder subdivisions covered by this article.

Synonyms

     Dysplasia of Nails With Hypodontia

Information on the following diseases can be found in the Related
Disorders section of this report:

     Rapp-Hodgkin's Syndrome
     Nail Dystrophy-Deafness Syndrome
     Hidrotic Ectodermal Dysplasias
     Anhidrotic Ectodermal Dysplasias

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 of this report.

Tooth and Nail Syndrome is a rare genetic, non-progressive disorder of
the fetal ectodermal germ cell layer.  The exact genetic and biochemical
defects causing this disorder are not understood and are thought to vary
between those affected.

Symptoms

Major symptoms of Tooth and Nail Syndrome may include lack of development of
mandibular incisors, second molars, maxillary canines, and other permanent
teeth.  Abnormal growth of nails on the hands and feet also occurs.

Causes

The exact cause of Tooth and Nail Syndrome is not known, although it is
thought to be inherited as an autosomal dominant trait.  (Human traits
including the classic genetic diseases, are the product of the interaction of
two genes for that condition, one received from the father and one from the
mother.  In dominant disorders, a single copy of the disease gene (received
from either the mother or father) will be expressed "dominating" the normal
gene and resulting in appearance of the disease.  The risk of transmitting
the disorder from affected parent to offspring is 50% for each pregnancy
regardless of the sex of the resulting child.)

Affected Population

Tooth and Nail Syndrome affects males and females in equal numbers.  It is
frequently found in high concentrations among Canadian Dutch Mennonite
populations.

Related Disorders

There are fifty-two Syndromes associated with Ectodermal Dysplasia.  Symptoms
of the following disorders are the most closely related to those of Tooth and
Nail Syndrome.  Comparisons may be useful for a differential diagnosis:

Rapp-Hodgkin's Syndrome is an inherited disorder in which the sweat
glands are affected as well as the teeth and nails.  (For more information on
this disorder, choose Ectodermal Dysplasia as your search term in the Rare
Disease Database).

Nail Dystrophy-Deafness Syndrome is an inherited disorder in which the
hearing is affected as well as the teeth and nails.

Hidrotic Ectodermal Dysplasias is an inherited disorder in which the
teeth are not affected as they are in Tooth and Nail Syndrome.

Anhidrotic Ectodermal Dysplasias is an inherited disorder which affects
the oil secreting (sebaceous) glands in the skin and hair shafts.  Syndromes
with this pattern tend to be more severe.  (For more information on these
disorders, choose Ectodermal Dysplasias as your search term in the Rare
Disease Database).

Therapies:  Standard

Treatment of Tooth and Nail Syndrome with the use of dentures may be helpful.
Genetic counseling may be of benefit for patients and their families.  Other
treatment is symptomatic and supportive.

Therapies:  Investigational

This disease entry is based upon medical information available through
October 1989.  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 Tooth and Nail Syndrome, please contact:

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

     National Foundation for Ectodermal Dysplasias
     219 E. Main St.
     Mascoutah, IL  62258
     (618) 566-2020

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

For genetic information and genetic counseling referrals:

     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

This Rare Disease Database entry is based upon outlines prepared by medical
and dental students (1984-1986) at the Medical College of Virginia for their
course in human genetics, and the following articles:

MENDELIAN INHERITANCE IN MAN, 7th ed.:  Victor A. McKusick; Johns Hopkins
University Press, 1986.  Pp.722.