eMedicine Specialties > Dermatology > Diseases of the Subcutaneous Tissue

Adiposis Dolorosa: Treatment & Medication

Author: Marjan Yousefi, MD, Department of Dermatology, Geisinger Medical Center
Coauthor(s): Tammie Ferringer, MD, Teaching Staff, Departments of Dermatology and Pathology, Geisinger Medical Center; Nada MacAron, MD, Staff Physician, Department of Pathology, Emory University School of Medicine
Contributor Information and Disclosures

Updated: May 15, 2009

Treatment

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:

  • Corticosteroids: 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
  • Anesthetics: 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.
  • Analgesics: Traditional analgesics, such as nonsteroidal anti-inflammatory drugs, have a poor effect. The lipomas are unresponsive to analgesics, and paracetamol and dextropropoxyphene are the first choices. Localized pain may sometimes be treated with a cortisone/anesthetic injection, alternatively with sterile water given intracutaneously or more deeply.
  • Others medications
    • 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

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.

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.

Diet

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

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.

Medication

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. However, in 2 reported cases, INF alfa-2b induced long-term relief of pain in 2 patients with Dercum disease (adiposis dolorosa) and chronic hepatitis C (see Medical Care).

Corticosteroids

These agents have anti-inflammatory properties and cause profound and varied metabolic effects. They modify the body's immune response to diverse stimuli.


Prednisone (Meticorten, Orasone, Deltasone, Sterapred)

May provide pain relief. Caution because the adverse effects may outweigh the benefits. Immunosuppressant for treatment of autoimmune disorders; may decrease inflammation by reversing increased capillary permeability and suppressing PMN activity. Stabilizes lysosomal membranes and also suppresses lymphocyte and antibody production.

Adult

20 mg PO qd; taper over several wk as condition improves

Pediatric

Not established

Coadministration with estrogens may decrease clearance; concurrent use with digoxin may cause digitalis toxicity secondary to hypokalemia; phenobarbital, phenytoin, and rifampin may increase metabolism of glucocorticoids (consider increasing maintenance dose); monitor for hypokalemia with coadministration of diuretics

Documented hypersensitivity; viral, fungal, connective tissue, or tubercular skin infections; peptic ulcer disease; hepatic dysfunction; GI disease

Pregnancy

B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals

Precautions

Abrupt discontinuation of glucocorticoids may cause adrenal crisis; hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, myasthenia gravis, growth suppression, and infections may occur with glucocorticoid use

Anesthetics

Lidocaine is reported to provide pain relief for 10 hours to several months.18,19 The exact mechanism of action is uncertain, and whether it is a central effect or due to its effect on blood flow remains to be elucidated. Cardiac monitoring is required. This should be considered an investigational therapy.


Lidocaine (Dilocaine, Xylocaine)

Decreases permeability to sodium ions in neuronal membranes. Results in inhibition of depolarization, blocking transmission of nerve impulses.

Adult

400 mg IV over 15 min qod

Pediatric

Not established

Coadministration with cimetidine or beta-blockers increases toxicity; coadministration with procainamide and tocainide may result in additive cardiodepressant action; may increase effects of succinylcholine

Documented hypersensitivity to amide-type local anesthetics; avoid in Adams-Stokes syndrome and Wolf-Parkinson-White syndrome; avoid in severe sinoatrial, AV, or intraventricular block, if artificial pacemaker not in place

Pregnancy

B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals

Precautions

Use a solution without preservatives; caution in heart failure, hepatic disease, hypoxia, hypovolemia or shock, respiratory depression, and bradycardia; may increase risk of CNS and cardiac adverse effects in elderly patients; high plasma concentrations may cause seizures, heart block, and AV conduction abnormalities

Analgesics

Pain control is essential for quality patient care, and it ensures patient comfort.


Acetaminophen (Feverall, Tempra, Aspirin Free Anacin, Tylenol)

Drug of choice for pain in patients with documented hypersensitivity to aspirin or NSAIDs, with upper GI tract disease, or who are taking oral anticoagulants.

Adult

650 mg PO q6h

Pediatric

Not established

Rifampin can reduce analgesic effects; coadministration with barbiturates, carbamazepine, hydantoins, and isoniazid may increase hepatotoxicity

Documented hypersensitivity; G-6-PD deficiency

Pregnancy

B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals

Precautions

Hepatotoxicity possible in persons with chronic alcoholism following various dose levels; severe or recurrent pain or high or continued fever may indicate a serious illness; acetaminophen is contained in many OTC products and combined use with these products may result in cumulative doses exceeding recommended maximum dose


Propoxyphene (Darvocet)

Drug indicated for mild to moderate pain.

Adult

65 mg PO q4h; not to exceed 390 mg/d

Pediatric

Not available

May increase serum concentrations of MAOIs, tricyclic antidepressants, carbamazepine, phenobarbital, and warfarin

Pregnancy

D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus

Precautions

Caution in patients dependent on opiates, substitution may result in acute opiate withdrawal symptoms; caution in severe renal or hepatic dysfunction, schedule IV narcotics

More on Adiposis Dolorosa

Overview: Adiposis Dolorosa
Differential Diagnoses & Workup: Adiposis Dolorosa
Treatment & Medication: Adiposis Dolorosa
Follow-up: Adiposis Dolorosa
References

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].

Further Reading

Keywords

adiposis dolorosa, Dercum's disease, Dercum disease, painful lipoma, fatty tissue rheumatism, juxta-articular adiposis dolorosa (occurs around the knees)

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, Teaching Staff, 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, and American 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.

Medical Editor

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.

Pharmacy Editor

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.

Managing Editor

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: Nothing to disclose.

CME Editor

Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University
Catherine 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.

 
 
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