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Disease Information (full text)

Dercum Disease

Synonyms | Disorder Subdivisions | General Discussion | Symptoms | Causes | Affected Population | Related Disorders | Standard Therapies | Investigational Therapies | Resources | References

Copyright (C) 1987, 1989, 1998 National Organization for Rare Disorders, Inc.

**IMPORTANT**
It is possible that the main title of the report (Dercum Disease) is not the name you expected. Please check the SYNONYMS listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

Synonyms
Adiposis Dolorosa
Juxta-Articular Adiposis Dolorosa


Disorder Subdivisions:


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 detailed information about this disorder, please contact your personal physician and/or the agencies listed in the "Resources" section this report.

Dercum Disease is a rare disorder characterized by pressure of fatty deposits on nerves, resulting in weakness and pain. This disorder usually occurs in obese females between the ages of 45 and 60. Various areas of the body may swell for no apparent reason. The swelling may disappear without treatment, leaving hardened tissue or pendulous skin folds. In some cases, affected individuals may also experience depression, lethargy, and/or confusion. Dercum Disease is thought to be inherited as an autosomal dominant genetic trait.
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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 areas 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 affected individuals may experience depression, lethargy, and/or confusion.
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Causes
Dercum Disease is thought to be inherited as an autosomal dominant genetic trait. Human traits, including the classic genetic diseases, are the product of the interaction of two genes, 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 other normal gene and resulting in the appearance of the disease. The risk of transmitting the disorder from affected parent to offspring is 50 percent for each pregnancy regardless of the sex of the resulting child. The risk is the same for each pregnancy.
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Affected Population
Dercum Disease, which was originally described in the medical literature in 1892 (F.X. Dercum), is approximately five times more frequent in females than males. The disorder primarily affects obese females between the ages of 45 and 60 years. In some cases, Dercum Disease may affect individuals of normal weight. According to the medical literature, the disease has been reported in several individuals within two multigenerational families (kindreds).
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Related Disorders
Symptoms of the following disorder may be similar to those of Dercum Disease. 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. In addition, in some cases, arthritis may be progressive.

Standard Therapies
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 may 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 affected individuals to cope with long-term intense pain. Other treatment is symptomatic and supportive.

Investigational Therapies


This disease entry is based upon medical information available through February 1998. 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, Inc.(NORD)
P.O. Box 8923
New Fairfield, CT 06812-8923
Telephone: (203) 746-6518
Fax: (203) 746-6481
Toll free: (800) 999-6673
TDD: (203) 746-6927
e-mail: orphan@nord-rdb.com
Home Page: http://www.nord-rdb.com/~orphan

NIH/National Institute of Diabetes, Digestive and Kidney Diseases
Building 31 Rm 9A04
31 Center Dr
Bethesda MD 20892-2560
(301) 496-3583



American Chronic Pain Association
P.O. Box 850
Rocklin CA 95677
(916) 632-0922
e-mail: ACPA@Pacbell.net
Home Page: http://www.theacpa.org



Dercum's Connection
712 Lincoln Avenue
Leesburg FL 34748
(352) 728-4367







References
ONLINE MENDELIAN INHERITANCE IN MAN (OMIM). Victor A. McKusick, Editor; Johns Hopkins University, Last Edit Date 3/11/94. Entry Number 103200.

ADIPOSIS DOLOROSA (DERCUM’S DISEASE): 10-YEAR FOLLOW-UP. S. Brodovsky et al.; Ann Plast Surg (Dec 1994; 33(6)). Pp. 664-68.

DERCUM’S DISEASE (ADIPOSIS DOLOROSA). TREATMENT OF THE SEVERE PAIN WITH INTRAVENOUS LIDOCAINE. P. Petersen et al.; Pain (Jan 1987; 28(1)). Pp. 77-80.

A CASE OF ADIPOSIS DOLOROSA: LIPID METABOLISM AND HORMONE SECRETION: A. Taniguchi et al.; Int J Obes (1986; 10(4)). Pp. 277-81.

DERCUM'S DISEASE (ADIPOSIS DOLOROSA). A CASE REPORT AND REVIEW OF THE LITERATURE: T.J. Bonatus et al.; Clin Orthop (April 1986; 205). Pp. 251-53.

AUTOSOMAL DOMINANT INHERITANCE IN ADIPOSIS DOLOROSA (DERCUM’S DISEASE). J.M. Cantu et al.; Humangenetik (1973; 18). Pp. 89-91.

THREE CASES OF A HITHERTO UNCLASSIFIED AFFECTION RESEMBLING IN ITS GROSSER ASPECTS OBESITY, BUT ASSOCIATED WITH SPECIAL NERVOUS SYMPTOMS: ADIPOSIS DOLOROSA. F.X. Dercum; Am J Med Sci (1892; 104). Pp. 521-35.
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The National Organization for Rare Disorders, Inc. and NORD
are registered service marks of
The National Organization for Rare Disorders, Inc.
P.O. Box 8923, New Fairfield, CT 06812-8923
Phone: (203) 746-6518 Fax: (203) 746-6481 Toll-Free: (800) 999-6673