Adiposis Dolorosa Treatment & Management

  • Author: Marjan Yousefi, MD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Nov 19, 2010
 

Medical Care

Traditional management of Dercum disease (adiposis dolorosa) has been largely unsatisfactory relying on weight reduction and surgical excision of particularly troublesome lesions. Even at the present time, no known drug can change the course of the disease, and available treatments are only symptomatic.

Nonpharmacological approaches for Dercum disease (adiposis dolorosa) may be used as adjuncts to pharmacologic treatments. Some of these include acupuncture, cognitive behavioral therapy, hypnosis, and biofeedback.[17]

The pharmacological treatments include the following:

  • Prednisone, 20 mg daily, has been reported to provide some pain relief.[2] However, in one case, the induction of disease was associated with high-dose corticosteroids.[4]
  • Intravenous lidocaine, 400 mg over 15 minutes every other day, has been reported to provide pain relief for 10 hours to several months.[18, 19] The exact mechanism of action is uncertain and remains to be elucidated as to whether it is a central effect or due to its effect on blood flow. Long-term intravenous lidocaine therapy has been associated with neurotoxicity.
  • Traditional analgesics, such as nonsteroidal anti-inflammatory drugs, have a poor effect. The lipomas are unresponsive to analgesics, and acetaminophen combined with an opioid analgesic is the first choice. Localized pain may sometimes be treated with a cortisone/anesthetic injection, alternatively with sterile water given intracutaneously or more deeply.

Others medications are as follows:

  • Because of troublesome swelling of the fingers, some patients may require diuretics.
  • In 2 reported cases of Dercum disease (adiposis dolorosa), interferon (INF) alfa-2b induced long-term relief of pain in 2 patients with adiposis dolorosa and chronic hepatitis C. The analgesic effect of IFN therapy was unexpected and occurred 3 weeks after treatment with 3 million units, 3 times per week, for 6 months. Whether the mechanism of pain relief with IFN is related to its antiviral effect, to the production of endogenous substances (eg, endorphins produced by IFN), or to the interference of INF with interleukin 1 and tumor necrosis factor-alpha cytokine production, which are involved in cutaneous hyperalgesias, remains unclear.[20]
  • Two Dercum disease (adiposis dolorosa) case reports have described pain relief with daily intake of oral mexiletine, an antiarrhythmic.[19, 21]
  • Singal et al reported improvement of a patient's Dercum disease (adiposis dolorosa) while on infliximab, with and without methotrexate, for ankylosing spondylitis. The patient experienced recurrent weight gain and lipoma pain with discontinuation of these medications.[22]
  • Desai et al reported on treatment with a lidocaine (5%) patch,[23] and Lange et al reported on successful therapy with pregabalin with manual lymphatic drainage.[24]
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Surgical Care

  • Liposuction
    • Liposuction is regarded as a supportive treatment for Dercum disease (adiposis dolorosa). Any skeletal pain is not affected. A significant initial reduction of pain and an improved quality of life is seen; these effects decrease over time.
    • Liposuction is indicated for patients with general lower-body fat or more localized large deposits of fat at the knees, on the arms, on the thighs, or on the stomach as opposed to those with general diffuse pain. In those patients, liposuction is considered a risky operation, requiring about a week of care in the plastic surgery department.
  • Surgical operation: Excision of isolated painful lipomas that are pressing and causing numbness and tingling, while not preventive, is useful in ameliorating local symptoms of pain.
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Consultations

  • Psychiatrist: Depression and other psychosomatic symptoms are associated with Dercum disease (adiposis dolorosa). Many patients find they are misjudged and require psychological support.
  • Rheumatologist: A rheumatologic consultation is warranted to rule out osteoarthritis and fibromyalgia.
  • Endocrinologist: An endocrinologic etiology, such as hypothyroidism and Cushing syndrome, should be ruled out.
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Diet

Experience shows that lasting weight reduction by changing the diet is difficult to achieve and does not appreciably affect the pain.

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Activity

Light physical activity may worsen symptoms because of the stiffness experienced after periods of rest and minimal activity. Patients should avoid monotonous, static work and physical and psychological stress.

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Contributor Information and Disclosures
Author

Marjan Yousefi, MD  Department of Dermatology, Geisinger Medical Center

Marjan Yousefi, MD is a member of the following medical societies: American Academy of Dermatology and Phi Beta Kappa

Disclosure: Nothing to disclose.

Coauthor(s)

Tammie Ferringer, MD  Dermatopathology Section Head, Dermatopathology Fellowship Director, Departments of Dermatology and Pathology, Geisinger Medical Center

Tammie Ferringer, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Society of Dermatopathology, and International Society of Dermatopathology

Disclosure: Nothing to disclose.

Nada Macaron, MD  Staff Physician, Department of Pathology, Emory University School of Medicine

Nada Macaron, MD, is a member of the following medical societies: College of American Pathologists and United States and Canadian Academy of Pathology

Disclosure: Nothing to disclose.

Specialty Editor Board

Catharine Lisa Kauffman, MD, FACP  Georgetown Dermatology and Georgetown Dermpath

Catharine Lisa Kauffman, MD, FACP is a member of the following medical societies: American Academy of Dermatology, American Medical Association, Royal Society of Medicine, Society for Investigative Dermatology, and Women's Dermatologic Society

Disclosure: Nothing to disclose.

David F Butler, MD  Professor of Dermatology, Texas A&M University College of Medicine; Chair, Department of Dermatology, Director, Dermatology Residency Training Program, Scott and White Clinic, Northside Clinic

David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Association of Military Dermatologists, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Rosalie Elenitsas, MD  Herman Beerman Associate Professor of Dermatology, University of Pennsylvania School of Medicine; Director, Penn Cutaneous Pathology Services, Department of Dermatology, University of Pennsylvania Health System

Rosalie Elenitsas, MD is a member of the following medical societies: American Academy of Dermatology and American Society of Dermatopathology

Disclosure: Lippincott Williams Wilkins Royalty Textbook editor; DLA Piper Consulting fee Consulting

Catherine M Quirk, MD  Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania

Catherine M Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD  Director, Department of Dermatology, Geisinger Medical Center

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

References
  1. Dercum FX. Three cases of a hitherto unclassified affection resembling in its grosser aspects obesity, but associated with special symptoms: adiposis dolorosa. Am J Med Sci. 1892;104:521-35.

  2. Palmer ED. Dercum's disease: adiposis dolorosa. Am Fam Physician. Nov 1981;24(5):155-7. [Medline].

  3. Brodovsky S, Westreich M, Leibowitz A, Schwartz Y. Adiposis dolorosa (Dercum's disease): 10-year follow-up. Ann Plast Surg. Dec 1994;33(6):664-8. [Medline].

  4. Greenbaum SS, Varga J. Corticosteroid-induced juxta-articular adiposis dolorosa. Arch Dermatol. Feb 1991;127(2):231-3. [Medline].

  5. Skagen K, Petersen P, Kastrup J, Norgaard T. The regulation of subcutaneous blood flow in patient with Dercum's disease. Acta Derm Venereol. 1986;66(4):337-9. [Medline].

  6. Lynch HT, Harlan WL. Hereditary Factors in Adiposis Dolorosa (Dercum's Disease). Am J Hum Genet. Jun 1963;15(2):184-90. [Medline].

  7. Cantu JM, Ruiz-Barquin E, Jimenez M, Castillo L, Macotela-Ruiz E. Autosomal dominant inheritance in adiposis dolorosa (Dercum's disease). Humangenetik. Mar 23 1973;18(1):89-91. [Medline].

  8. Campen R, Mankin H, Louis DN, Hirano M, Maccollin M. Familial occurrence of adiposis dolorosa. J Am Acad Dermatol. Jan 2001;44(1):132-6. [Medline].

  9. Leites SM, Davtian NK, Emanuel' VIa. [Pathophysiological characteristics of adipose tissue in Dercum's syndrome]. Patol Fiziol Eksp Ter. Jan-Feb 1972;16(1):47-51. [Medline].

  10. Blomstrand R, Juhlin L, Nordenstam H, Ohlsson R, Werner B, Engstrom J. Adiposis dolorosa associated with defects of lipid metabolism. Acta Derm Venereol. 1971;51(4):243-50. [Medline].

  11. Gamez J, Playan A, Andreu AL, et al. Familial multiple symmetric lipomatosis associated with the A8344G mutation of mitochondrial DNA. Neurology. Jul 1998;51(1):258-60. [Medline].

  12. Silvestri G, Ciafaloni E, Santorelli FM, et al. Clinical features associated with the A-->G transition at nucleotide 8344 of mtDNA ("MERRF mutation"). Neurology. Jun 1993;43(6):1200-6. [Medline].

  13. Stallworth JM, Hennigar GR, Jonsson HT Jr, Rodriguez O. The chronically swollen painful extremity. A detailed study for possible etiological factors. JAMA. Jun 24 1974;228(13):1656-9. [Medline].

  14. Amine B, Leguilchard F, Benhamou CL. Dercum's disease (adiposis dolorosa): a new case-report. Joint Bone Spine. Mar 2004;71(2):147-9. [Medline].

  15. Freedberg IM, Eisen AZ, Wolff K et al, eds. Neoplasms of subcutaneous fat. In: Dermatology in General Medicine. Vol 1. 5th ed. New York, NY: McGraw-Hill; 1999:1348-9.

  16. Fagher B, Monti M, Nilsson-Ehle P, Akesson B. Fat-cell heat production, adipose tissue fatty acids, lipoprotein lipase activity and plasma lipoproteins in adiposis dolorosa. Clin Sci (Lond). Dec 1991;81(6):793-8. [Medline].

  17. Campen RB, Sang CN, Duncan LM. Case records of the Massachusetts General Hospital. Case 25-2006. A 41-year-old woman with painful subcutaneous nodules. N Engl J Med. Aug 17 2006;355(7):714-22. [Medline].

  18. Iwane T, Maruyama M, Matsuki M, Ito Y, Shimoji K. Management of intractable pain in adiposis dolorosa with intravenous administration of lidocaine. Anesth Analg. Mar-Apr 1976;55(2):257-9. [Medline].

  19. Petersen P, Kastrup J. Dercum's disease (adiposis dolorosa). Treatment of the severe pain with intravenous lidocaine. Pain. Jan 1987;28(1):77-80. [Medline].

  20. Gonciarz Z, Mazur W, Hartleb J, et al. Interferon alfa-2b induced long-term relief of pain in two patients with adiposis dolorosa and chronic hepatitis C. J Hepatol. Dec 1997;27(6):1141. [Medline].

  21. Steiner J, Schiltz K, Heidenreich F, Weissenborn K. [Lipomatosis dolorosa--a frequently overlooked disease picture]. Nervenarzt. Feb 2002;73(2):183-7. [Medline].

  22. Singal A, Janiga JJ, Bossenbroek NM, Lim HW. Dercum's disease (adiposis dolorosa): a report of improvement with infliximab and methotrexate. J Eur Acad Dermatol Venereol. May 2007;21(5):717. [Medline].

  23. Desai MJ, Siriki R, Wang D. Treatment of pain in Dercum's disease with Lidoderm (lidocaine 5% patch): a case report. Pain Med. Nov 2008;9(8):1224-6. [Medline].

  24. Lange U, Oelzner P, Uhlemann C. Dercum's disease (Lipomatosis dolorosa): successful therapy with pregabalin and manual lymphatic drainage and a current overview. Rheumatol Int. Nov 2008;29(1):17-22. [Medline].

  25. Haddad D, Athmani B, Costa A, Cartier S. [Dercum's disease: a severe complication in a rare disease. A case report]. Ann Chir Plast Esthet. Jun 2005;50(3):247-50. [Medline].

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