Adiposis Dolorosa Clinical Presentation

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

History

  • Presentation
    • Previously healthy women notice lumps or previously present lumps start growing. They describe pain and discomfort in the region of the lumps associated with weakness.
    • Before the onset of the disease, the patient is usually only slightly obese, but, in a short time, the patient becomes overweight.
    • The pain increases with the increase in fatty tissue and in connection to menstruation.
  • Types and location
    • The painful lipomas have been reported to occur in any location, except in the head and the neck.[13]
    • Different types can be identified according to the spread of pain.
      • Type I, or the juxta-articular type, with painful folds of fat on the inside of the knees and/or on the hips, in rare cases only evident in upper-arm fat
      • Type II, or the diffuse, generalized type, where widespread pain from fatty tissue is found, apart from that of type I, also often in the dorsal upper-arm fat, in the axillary and gluteal fat, in the stomach wall, in dorsal fat folds, and on the soles of the feet
      • Type III, or the lipomatosis, nodular type, with intense pain in and around multiple lipomas, sometimes in the absence of general obesity; lipomas are approximately 0.5-4 cm, soft, and attached to the surrounding tissue (Histologically, these are not always encapsulated. Some have been classified as angiolipomas.)
  • Type of pain
    • The pain varies from discomfort on palpation to excruciating, paroxysmal spontaneous attacks.[3]
    • The pain can be aching, burning, or stabbing, often described by the patient as "it hurts everywhere."
    • The pain is usually symmetrical; however, it can become localized to the thighs, the knees, or the upper extremities.
    • Pain can be felt in the skeletal system and in the fat.
  • Hyperalgesia is found by light pressure and touch in the fatty tissue below the skin and is made worse by tightly fitting clothes or showering. The pain is temperature and weather dependent; it decreases in dry heat and when pressure is high. Hot baths can have a positive but short-term effect in the relief of pain, but some patients do not tolerate heat. Estrogen replacement at menopause does not reduce the pain.
  • Other symptoms, with variable incidence, include the following:
    • The fingers have a tendency to swell up, fumble, and tingle, and they can be numb (paresthesias), in addition to secondary median nerve compression.
    • General tiredness similar to the symptoms of chronic fatigue syndrome may be present. Light physical activity and poor sleep aggravate the tiredness.
    • A tendency to bruise, possibly secondary to the formation of delicate vessels in fat deposits, may be present. Coagulation test results are normal.
    • Morning stiffness and stiffness after resting may occur.
    • Headaches (eg, tension headaches, classic migraine, neck headaches) may occur. Also, pain in the jaw and the eyes due to retrobulbar fatty tissue may be present.
    • Cognitive dysfunction, with concentration and memory problems, may be present.
    • Bouts of depression (atypical depression, possibly latent) may occur; this finding is not associated with the onset of the disease.
    • Feeling hot affects a small number of patients, with recurring high temperatures of 37.5-39°C for weeks at a time associated with worsening of pain.
    • Patients may become susceptible to infection, which is possibly due to the presence of fat. Pain is exacerbated with infections.
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Physical

Dercum disease (adiposis dolorosa) symptoms are almost always out of proportion to the physical findings, which include the following:

  • Dercum disease (adiposis dolorosa) patients are usually 50% over the normal weight for their age. In some patients, only localized fat, without general obesity, is present.
  • Lipomas are multiple, painful, symmetrically distributed, fatty deposits that are either diffuse or localized. The abdominal region and the lower extremities are common sites, especially around the knees. The ankle is an uncommon site of involvement.[14]
  • Hyperalgesia is found in the fatty tissue below the skin on light pressure and touch.
  • Other findings include acral swelling, bruises, and telangiectasias.
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Causes

  • The cause is unknown.
  • High-dose corticosteroids were the cause in a reported case.
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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

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

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

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  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].

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  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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