Adiposis Dolorosa (Dercum Disease) Treatment & Management

Updated: Aug 25, 2021
  • Author: Laura F McGevna, MD; Chief Editor: Dirk M Elston, MD  more...
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Treatment

Approach Considerations

There is no curative treatment. Therapies should be individualized to specific symptoms in each patient, with the overall goal to relieve symptoms and improve quality of life. [1] Therapeutic options that have been suggested by case reports include analgesics, liposuction, lipectomy, manual lymphatic drainage, and minimal incision technique. [1, 45] Physicians may also consider ketamine, electrostimulation, and perineural injections. [46] Interdisciplinary team management is crucial, as delayed diagnosis is associated with high morbidity. Pharmacist input on treatment options, patient education and monitoring, and coordinating follow-up are important aspects of care. [1]

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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 in current practice, no known drug can change the course of the disease, and available treatments are only symptomatic.

Nonpharmacological approaches for Dercum disease may be used as adjuncts to pharmacologic treatments. Some of these include acupuncture, cognitive behavioral therapy, hypnosis, and biofeedback. [47, 48]

Pharmacological treatments

Prednisone in doses no higher than 20 mg/day has been reported to provide some pain relief. [8] However, in one case, the induction of disease was associated with high-dose corticosteroids. [14]

Intravenous lidocaine, 400 mg over 15 minutes every other day, has been reported to provide pain relief for 10 hours to several months. [49, 50] 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 and may potentially lower seizure threshold. [46]

Traditional analgesics, such as nonsteroidal anti-inflammatory drugs (NSAIDs), have traditionally been thought to have a poor effect. However, a large 2007 series by Herbst and Asare-Bediako concluded that 89% achieved relief when treated with an NSAID, as did 97% when treated with an opiate. [7] Acetaminophen is a reasonable first-line choice. Localized pain may sometimes be treated with a cortisone/anesthetic injection, alternatively with sterile water given intracutaneously or more deeply.

Other medications

Low-dose d-amphetamine was found to anecdotally improve pain in patients. Two cases, a 55-year-old man and a 52-year-old woman, described treatment with d-amphetamine to improve lymphatic function through the sympathetic nervous system; it led to weight loss and improvement in Dercum disease and resolution of hepatic steatosis in the man and resolution of hepatic lipomas in the woman. [51]

Ketamine infusions have been used to treat other chronic pain syndromes, including chronic cancer pain, complex regional pain syndrome, fibromyalgia, migraine, ischemic pain, and neuropathic pain. A case report of a 53-year-old man diagnosed with Dercum disease found that ketamine infusions (500 mg of ketamine in a 500-mL bag of 0.9% NaCl) reduced the patient's pain to 0 of 10 postprocedurally and was sustained for 3 months. [46]

Because of troublesome swelling of the fingers, some patients may require diuretics.

In two reported cases of Dercum disease, interferon (INF) alfa-2b induced long-term relief of pain in two patients with adiposis dolorosa and chronic hepatitis C. The analgesic effect of INF 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 INF is related to its antiviral effect, to the production of endogenous substances (eg, endorphins produced by INF), or to the interference of INF with interleukin (IL)–1 and tumor necrosis factor-alpha cytokine production, which are involved in cutaneous hyperalgesias, remains unclear. [52]

Two Dercum disease case reports have described pain relief with daily intake of oral mexiletine, an antiarrhythmic. [50, 53]

Singal et al reported improvement of a patient's Dercum disease while on infliximab, with and without methotrexate, for ankylosing spondylitis. The patient experienced recurrent weight gain and lipoma pain with discontinuation of these medications. [54]

Desai et al reported on treatment with a lidocaine (5%) patch, [55] and Lange et al reported on successful therapy with pregabalin with manual lymphatic drainage. [56] Calcium channel modulators such as oxcarbazepine (and pregabalin) have also been tried. [46]

Metformin has been used with success in a patient with adiposis dolorosa and associated pain. [26] It is thought that the drug may have the capacity to favorably alter the cytokine milieu, impacting such mediators as tumor necrosis factor (TNF), IL-1, and leptin. [6, 26] In the report by Labuzek et al, each variable was affected moderately by the drug, and it was concluded that the effect of reduction of the inflammatory mediators is additive. Nonetheless, they concluded that other phenomena must contribute to the effects (eg, modulation of synaptic plasticity, activation of microglia). [26]

It should be noted that a study on cytokines in patients with adiposis dolorosa revealed there is no significant difference between these patients and controls with regard to TNF, leptin, IL-1, and most other mediators. However, patients with disease did demonstrate significantly lower levels of macrophage inhibitory protein-1 beta and higher levels of IL-13 and lower levels of fractalkine, an adipokine whose receptors are characteristically up-regulated in prolonged neuropathic pain. [13, 57]

Nonpharmacologic modalities

Hypobaric pressure therapy has been considered as a method to treat pain associated with edema. A pilot study focused on hypobaric pressure therapy in patients with Dercum disease using a cyclic altitude conditioning system, which reported decreased pain after 5 days of therapy. [58]

Manual lymphatic twice weekly massage has been used to treat the obstructive symptoms that are seen with lipomatous growths in Dercum disease. However, it has also been noted that some patients found massage to worsen the progression of lipomatous growths. [46]

Acupuncture can be beneficial in chronic pain relief, but there are no research studies or case reports regarding acupuncture in the management of Dercum disease. [46]

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Surgical Care

Liposuction  [59]

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. [60]

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. Additionally, liposuction is contraindicated in recurrent lipomas. [59]

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 in the short term. [46] A case report detailed resection with interval application of wound vacuum-assisted closure combined with delayed closure with split-thickness skin grafting as an alternative for large, exophytic adiposis dolorosa that affected patient mobility and was not primarily for pain relief. [61]

In 2020, Cuellar-Barboza et al published the use of a minimal-incision technique to treat Dercum disease. [45] This was described as a simple surgical technique to remove lesions on a 46-year-old woman. A 4-mm punch was inserted into the top of the cutting surface through the center of the lesion. Dissection to extract the lipoma (either whole or piecemeal), irrigation with saline solution, and revision of the area were the subsequent steps taken to assure extirpation. One to two interrupted cutaneous sutures closed the incision sites. The authors found that at a 6-month follow-up, the patient's quality of life had significantly improved and she had no recurrence in pain.

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Therapeutic Procedures

Electrocutaneous stimulation, perineural injection therapy/prolotherapy, and intralesional deoxycholic acid have also been suggested to treat the pain associated with Dercum disease (adiposis dolorosa).

Transcutaneous frequency rhythmic electrical modulation system (FREMS) treatment of 4 cycles of 30 minutes each for 6 months decreased pain in patients with Dercum disease. Another cutaneous electrostimulation modality that has been used for chronic cancer pain management is the MC5-A Calmare, which can be considered for treatment of Dercum disease. However, this modality is limited by insurance coverage, access to this machine, operator training, and reproducibility of electrode placement, an important step to achieve pain relief. [46]

Perineural injection therapy (PIT)/prolotherapy is another modality that can be considered, although there have been no research studies or case reports published on its use in Dercum disease. PIT involves a dextrose solution injection into tissues surrounding an inflamed nerve to reduce neuropathic inflammation; pain relief is usually immediate but several treatments are required to ensure a lasting benefit. Dextrose prolotherapy has been used to treat chronic musculoskeletal pain; however, the lack of insurance coverage and access to physicians who perform the procedure make this option unavailable to most patients. [46]

The off-label use of intralesional deoxycholic acid, an endogenous secondary bile acid that assists in the breakdown of dietary fat in the gut, was described in 2019 to treat Dercum disease in a 46-year-old White man after several medical (antidepressants, narcotics, pregabalin, metformin), procedural (intralesional 40-mg/mL triamcinolone), and surgical therapies (lipoma excisions) failed to provide adequate relief. The patient reported overall decreased pain and reduced size of lesions at a 3-month clinic follow-up. [31]

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Consultations

The following consultations may be warranted:

  • Pain medicine specialist: Pain may be debilitating. [1]
  • Psychiatrist: Depression and other psychosomatic symptoms have been 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.
  • Surgeon: Larger lipomas may need to be removed surgically with dermolipectomy.

 

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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

Extended sedentary periods should be avoided as a return to even light physical activity may aggravate symptoms because of the stiffness experienced. Patients should avoid monotonous, static work and physical and psychological stress.

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