$Unique_ID{BRK03755}
$Pretitle{}
$Title{Frey's Syndrome}
$Subject{Frey's Syndrome von Frey's Syndrome Baillarger's Syndrome Dupuy's
Syndrome Auriculotemporal Syndrome Salivosudoriparous Syndrome
Sweating-Gustatory Syndrome}
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$Log{}

Copyright (C) 1986, 1987, 1988 National Organization for Rare Disorders,
Inc.

101:
Frey's Syndrome

** IMPORTANT **
It is possible that the main title of the article (Frey'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

     von Frey's Syndrome
     Baillarger's Syndrome
     Dupuy's Syndrome
     Auriculotemporal Syndrome
     Salivosudoriparous Syndrome
     Sweating-Gustatory Syndrome

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.


Frey's syndrome is characterized by flushing or sweating on one side of
the face when eating certain foods.  It results from injury to the parotid
gland, a large salivary gland near the ear.  Most patients tolerate the
condition, but some require treatment.

Symptoms

In Frey's Syndrome, the cheek and ear on one side of the face sweats and
becomes red whenever the patient eats hot, spicy, or very acidic food, or
chocolate.  The flushed part of the face becomes less sensitive to heat.  Over
time, a slightly larger area may become involved.  In men, the predominant
manifestation is sweating.  In women, the flushing predominates.

Causes

Frey's syndrome usually develops only after injury, surgery, or inflammation
of one of the parotid glands and the nerve supplying it.  The sweating and
flushing are due to abnormal regeneration of parasympathetic nerve fibers
which have been injured, so the sweat glands and blood vessels of the skin
which normally have a sympathetic innervation acquire a parasympathetic nerve
supply.  These aberrant parasympathetic nerves are stimulated when the
patient eats.

Related Disorders

Hyperhidrosis (excessive sweating) may affect the entire body or be confined
to certain areas of the body.  When the entire body is affected, the eccrine
sweat glands cause the problem, while the localized type is produced by
apocrine sweat glands.  When Hyperhidrosis is localized, it usually affects
the palms, soles, underarms, the area between the breasts, or the groin.
(For more information, choose "hyperhidrosis" as your search term in the Rare
Disease Database.)

Therapies:  Standard

When Frey's Syndrome causes excessive discomfort, the patient should be
treated.  Procaine, a local anesthetic, may be injected into the
auriculotemporal nerve supplying the area in question.  Surgically, the nerve
projections near the ear and cheek may be modified.  Scopolamine cream may be
applied to the affected skin.

Therapies:  Investigational

The orphan drug Glycopurrolate is being developed by Robins Corporation
for patients with Frey's Syndrome.

This disease entry is based upon medical information available through
December 1988.  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 Frey's Syndrome, please contact:

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

     Association for Glycogen Storage Diseases
     Box 896
     Durant, IA  52747
     (319) 785-6038

     The National Arthritis and Musculoskeletal and Skin Diseases Information
     Clearinghouse
     Box AMS
     Bethesda, MD  20892
     (301) 495-4484