eMedicine Specialties > Dermatology > Diseases of the Subcutaneous Tissue

Adiposis Dolorosa: Differential Diagnoses & Workup

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

Differential Diagnoses

Lipodystrophy, Progressive
Proteus Syndrome

Other Problems to Be Considered

Like fibromyalgia, the diagnosis of Dercum disease (adiposis dolorosa) is made clinically, and both diseases include symptoms of a number of associated diseases. However, in Dercum disease (adiposis dolorosa), a relationship exists between pain and body weight, with pain in the fatty tissue and obesity being fundamental criteria for the diagnosis of Dercum disease (adiposis dolorosa). In addition, the pain is often more general and more severe than in fibromyalgia.

Madelung syndrome, also known as multiple symmetrical lipomatosis or benign symmetrical lipomatosis, is characterized by numerous, symmetrically distributed, nontender, poorly circumscribed lipomas, mainly around the neck, in the suboccipital region, on the proximal extremities, and on the upper part of the trunk. It is an idiopathic disease that affects middle-aged, nonobese men who are alcoholics. Neurologic involvement, particularly peripheral neuropathy, is considered a constitutive manifestation of this disease.15

Familial multiple lipomatosis belongs to the multiple lipoma syndromes. It is transmitted in an autosomal dominant fashion and often becomes apparent by the third decade of life. Patients may have up to hundreds of slowly growing, usually asymptomatic, subcutaneous lipomas of various sizes in widespread distribution. Patients with familial multiple lipomatosis are distinguished from patients with Dercum disease (adiposis dolorosa) by their lack of disabling pain.15

Proteus syndrome is characterized by lipomas, partial gigantism of the hands or the feet, hemihypertrophy, pigmented nevi, and other subcutaneous neoplasms (eg, hemangiomas, lymphangiomas, mesenchymomas).

Weber-Christian disease (nonspecific panniculitis), neurofibromatosis, Fröhlich syndrome, adenolipomatosis, lipodystrophia progressiva, Cushing syndrome, and osteoarthritis should be ruled out when evaluating patients with multiple subcutaneous tumors. Also, in a patient who is obese, myasthenia gravis should be considered.

Workup

Laboratory Studies

  • Results of hormonal studies to rule out Cushing syndrome, thyroid abnormalities, and other endocrinologic abnormalities are normal.
  • Dercum disease (adiposis dolorosa) Patients might have associated slight-to-moderate rises of cholesterol levels.
  • Erythrocyte sedimentation rate results can be slightly elevated.
  • Coagulation test results are normal.
  • In spite of obesity, hypertension and type 2 diabetes mellitus seldom occur.
  • An increase in certain active parameters is seen in the following: sedimentation rate; alpha-1-antitrypsin; orosomucoid (alpha-1-acid glycoprotein, an acute phase reactant); haptoglobin; and complement factors C3, C4, Clq, and Cls.4,5
  • The heat produced by the fat cells when measured with a microcalorie meter is approximately twice as high as that taken from people who are extremely overweight.
  • The ratio of monounsaturated fatty acid (16:1, 18:1) in the fatty tissue is greater than that of saturated fatty acid (14:1, 18:0) shown by a comparison with healthy people in controls.15,16
  • The levels of substance P in the cerebrospinal fluid is significantly lower compared with healthy weight-matched controls. However, the average in both cases is above the normal level. The level of the neuropeptide Y is on the lower side of normal, and B-endorphin is on the higher side (H. Brorson, B. Fagher, R. Ekman; unpublished data).

Imaging Studies

  • Ultrasonography and magnetic resonance imaging may aid in the diagnosis of Dercum disease (adiposis dolorosa).14

Histologic Findings

A review of histopathologic findings did not reveal any significant features that might distinguish Dercum disease (adiposis dolorosa) tumors from the common sporadic lipomas. Minor features that were detected include a slight accumulation of perivascular lymphocytes and plasma cells and extremely large fat cells compared with those of healthy controls of similar weight. The tumors can be encapsulated, or the fatty deposits can be diffuse.

More on Adiposis Dolorosa

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

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Keywords

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

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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, 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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