$Unique_ID{BRK03656}
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$Title{Dercum Disease}
$Subject{Dercum Disease Adiposis Dolorosa Juxta-articular Adiposis Dolorosa
Arthritis}
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Copyright (C) 1987, 1989 National Organization for Rare Disorders, Inc.

490:
Dercum Disease

** IMPORTANT **
It is possible the main title of the article (Dercum Disease) is not the
name you expected.  Please check the SYNONYMS listing on the next page to
find alternate names, disorder subdivisions, and related disorders covered
by this article.

Synonyms

     Adiposis Dolorosa
     Juxta-articular Adiposis Dolorosa

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

     Arthritis

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.

Dercum Disease is a painful disorder characterized by pressure of fatty
deposits on nerves leading to weakness.  This disorder usually occurs in
obese women between the ages of forty-five and sixty.  Various parts of the
body may swell for no apparent reason.  The swelling may disappear without
treatment leaving hardened tissue or pendulous skin folds.  Emotional
disturbances may occur in some cases.

Symptoms

Dercum Disease is characterized by pain and weakness caused by pressure on
nerves by abnormal fatty deposits near joints.  Swellings consisting of
irregularly shaped soft fatty tissue deposits may occur in many parts of the
body.  The knees, trunk, forearms and thighs are most commonly affected.
These deposits may spontaneously resolve leaving hardened tissue or pendulous
folds of skin.  Some patients may experience feelings of depression, but it is
not known whether these emotional feelings are a symptom of the disorder or a
response to the pain of the chronic illness.

Causes

The exact cause of Dercum Disease is not known.  Some physicians suggest it
may be genetic or it may be associated with an unidentified endocrine
abnormality.

Affected Population

Dercum Disease usually affects obese females between the ages of forty-five
and sixty years.  It has been recorded in more than one member of the same
family.  However, rare cases affecting men have been reported in the medical
literature, and it has been known to affect people of normal weight.

Related Disorders

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

Arthritis is defined as inflammation of a joint.  All forms of arthritis
are marked by some degree of pain and/or swelling as well as a wide variety
of other symptoms.  This disorder may be chronic or short-term; sometimes it
is progressive.  (For more information on rare forms of this disorder,
choose "Arthritis" as your search term in the Rare Disease Database and see
the Arthritis section of the Prevalent Health Conditions/Concerns area of
NORD Services).

Therapies:  Standard

Treatment of Dercum Disease is primarily directed at easing painful
episodes.  Surgical excision of fatty tissue deposits around joints may
temporarily relieve symptoms although recurrences often develop.
Intravenous infusions of the local anesthetic drug lidocaine can give
temporary relief from pain.  Additional treatments of lidocaine may be
necessary periodically to sustain the effect.  Another analgesic drug,
peroral mexiletine, may also be effective in eliminating pain for variable
periods of time.  Psychotherapy may be helpful for enabling patients to cope
with long-term intense pain.  Other treatment is symptomatic and supportive.

Therapies:  Investigational

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 Dercum Disease, please contact:

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

     National Digestive Diseases Information Clearinghouse
     Box NDDIC
     Bethesda, MD  20892
     (301) 468-6344

     American Chronic Pain Association, Inc.
     257 Haymaker Rd.
     Monroeville, PA  15146-1711
     (412) 856-9676

     National Chronic Pain Outreach Association
     8222 Wycliffe Ct.
     Manassas, VA  22110
     (703) 368-7357

References

MENDELIAN INHERITANCE IN MAN, 7th ed.:  Victor A. McKusick; Johns Hopkins
University Press, 1986.  P. 26.

A CASE OF ADIPOSIS DOLOROSA:  LIPID METABOLISM AND HORMONE SECRETION:
A. Taniguchi, et al.; Int J Obes (1986, issue 10 (4)).  Pp. 277-281.

DERCUM'S DISEASE (ADIPOSIS DOLOROSA).  A CASE REPORT AND REVIEW OF THE
LITERATURE:  T.J. Bonatus, et al.; Clin Orthop (April 1986, issue 205).  Pp.
251-253.

DERCUM'S DISEASE (ADIPOSIS DOLOROSA).  TREATMENT OF SEVERE PAIN WITH
INTRAVENOUS LIDOCAINE:  P. Petersen, et al.; Pain (January 1987, issue
28(1)).  Pp. 77-80.