$Unique_ID{BRK03751}
$Pretitle{}
$Title{Fox-Fordyce Disease}
$Subject{Fox-Fordyce Disease Apocrine Duct Occlusion Sweat Retention Disease
Hidradenitis Suppurativa Miliaria}
$Volume{}
$Log{}

Copyright (C) 1993 National Organization for Rare Disorders, Inc.

941:
Fox-Fordyce Disease

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

Synonyms

     Apocrine Duct Occlusion
     Sweat Retention Disease

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

     Hidradenitis Suppurativa
     Miliaria

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.

Fox-Fordyce Disease is a rare disorder that occurs almost solely in
women.  It is characterized by the development of intense itching usually in
the underarm area, the pubic area, and around the nipple of the breast.
Perspiration becomes trapped in the sweat gland and in the surrounding area
causing intense itching, inflammation, and enlargement of the glands.  Skin
in the area may become darkened and dry; raised patches develop.  Hair
follicles in the area dry out resulting in loss or breakage of hair.

Symptoms

Fox-Fordyce Disease is characterized by dry, darkened patches of skin in the
underarm, pubic, and nipple area of the body with raised patches of dried out
blisters.  Perspiration that becomes trapped in the gland or in the
surrounding skin produces intense itching, inflammation, and swelling of the
gland.  The disease can cause loss or breakage of hair follicles in these
areas.  The disorder almost solely effects women after puberty and around the
time of menstruation.

Causes

Fox-Fordyce Disease is caused by abnormal functioning of the sweat glands in
the underarm area, in the groin, and around the nipple area of the breast.
Perspiration becomes trapped in the gland or the surrounding area resulting
in inflammation, swelling, and intense itching.  The skin of the effected
area may have raised patches of dried out blisters, be darkened, and the hair
may become broken or fall out.

Researchers suspect that the disorder is related to hormonal conditions,
but they do not know which hormones are involved or why.

Affected Population

Fox-Fordyce Disease is a rare disorder that effects females almost
exclusively.  It usually begins after puberty.  The disorder may be more
severe at the time of menstruation and tends to disappear during pregnancy.

Related Disorders

Symptoms of the following disorders can be similar to those of Fox-Fordyce
Disease.  Comparisons may be useful for a differential diagnosis:

Hidradenitis Suppurativa is a chronic inflammatory enlargement of the
sweat glands with scarring that is associated with bacterial infection.
Nodules form under the skin that are similar to boils.  Swelling, pain, and
an accumulation of pus enlarge the gland until surgical removal is necessary.
(For more information on this disorder, choose "Hidradenitis Suppurativa" as
your search term in the Rare Disease Database).

Miliaria is a common occurrence and is usually known as "heat rash".  The
sweat gland is blocked and fluid is trapped in the surrounding area.  There
are various types.  However, they do not involve the hair follicle and do not
result in hair loss and inflammatory conditions.  Cooling the patient with
water or compresses or putting them in a cooler place usually results in the
disappearance of the rash.

Therapies:  Standard

In severe cases Fox-Fordyce Disease can be treated with surgical removal of
the inflamed gland.  Other treatments may involve the use of topical
application of a form of vitamin A.  However, this treatment is being
questioned as the use may cause cancer.

The drug clindamycin in an alcohol propylene glycol solution is another
form of treatment.

Therapies:  Investigational

The antiandrogen (cyproterone acetate) in combination with or without
estrogen therapy is being tested as a treatment for Fox-Fordyce Disease.
More study is needed to determine the long term safety and effectiveness of
this treatment.

This disease entry is based upon medical information available through
May 1993.  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 Fox-Fordyce Disease, please contact:

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

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

References

CLINICAL DERMATOLOGY, 2nd Ed.; Thomas P. Habif, M.D., Editor:  The C.V. Mosby
Company, 1990.  Pp. 139-140.

FOX-FORDYCE DISEASE:  SUCCESSFUL TREATMENT WITH TOPICAL CLINDAMYCIN IN
ALCOHOLIC PROPYLENE GLYCOL SOLUTION., R. Feldmann, et al.; Dermatology, 1992,
(issue 184 (4)).  Pp. 310-313.

THE THERAPEUTIC USES OF TOPICAL VITAMIN A ACID., J.R. Thomas, et al.; J
Am Acad Dermatol, May, 1981, (issue 4 (5)).  Pp. 505-513.