Adiposis Dolorosa (Dercum Disease) 

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

Overview

Practice Essentials

Diagnosis of Dercum disease (adiposis dolorosa) is made clinically and is a diagnosis of exclusion.[1] A multidisciplinary approach to treatment is recommended. Referrals should be made to the following specialties: pain medicine, surgery, and psychiatric care, with treatment focused on both pain relief and restoring normal appearance.[2] While classically a disease of adults, a rare case of a child with Dercum disease was reported in 2018.[3]

Background

Adiposis dolorosa is most commonly known as Dercum disease, but it is also known as Ander syndrome, morbus Dercum, adipose tissue rheumatism, adiposalgia, or lipomatosis dolorosa.[1] First described in 1892 by the American neurologist Francis Xavier Dercum at Jefferson Medical College in Philadelphia, Pennsylvania, Dercum disease (adiposis dolorosa) is an unusual progressive syndrome of unknown etiology characterized by multiple painful lipomas (see images below) that usually arise in adult life, most often affecting obese postmenopausal women.[4] Dercum disease is a subcutaneous adipose tissue disease that involves the adipose tissue and its fascia (ie, adipofascial disorders). Adipofascial disorders represent variations in the spectrum of obesity.[5]

Multiple painful lipomas. Courtesy of Waikato Dist Multiple painful lipomas. Courtesy of Waikato District Health Board and DermNet New Zealand (http://www.dermnetnz.org/assets/Uploads/dermal-infiltrative/w/dercum-1.jpg).

The onset of Dercum disease is insidious, but it has been described in at least one patient as having occurred after puerperal weight gain.[6] The pain is out of proportion to the physical findings and is often described by patients as "painful fat.” The pain increases with increases in fatty tissue and in connection with menstruation. Estrogen replacement at menopause has not been shown to reduce the pain.

Since its original description, the clinical spectrum has changed to include other components of Dercum disease to various degrees.[7] This is in addition to the painful nodular fatty deposits (which are often unaffected by weight loss). More recently, general obesity, easy fatigability and weakness (asthenia), and a wide variety of unexplained emotional disturbances, such as depression, confusion, and dementia, have been reported. This observation is why Dercum disease has been proposed to be relabeled as Dercum syndrome.[8]

Dercum disease has been classified by the World Health Organization (WHO) as a distinct entity. The National Organization of Rare Diseases (NORD) notes, "Dercum Disease is a rare disorder in which there are fatty deposits which apply pressure to the nerves, resulting in weakness and pain. Various areas of the body may swell for no apparent reason. The swelling may disappear without treatment, leaving hardened tissue or pendulous skin folds."

Criteria for diagnosis

In 1901, Roux and Vitaut first proposed the following four cardinal symptoms of Dercum disease, and these remain the standard for diagnosis of classic disease, although they have not been validated[9, 10, 11] :

  • Multiple, painful, fatty masses

  • Generalized obesity, usually in menopausal age

  • Asthenia

  • Neuropsychiatric disturbances, including emotional instability, depression, epilepsy, confusion, and dementia

As early as 1910, Stern noted that neuropsychiatric disturbances and asthenia did not accompany every case, and numerous case reports were subsequently described without all four cardinal features.[12] Therefore, some have lobbied for a “minimal definition” of adiposis dolorosa, which was proposed to include the following[13] :

  • Generalized obesity

  • Chronic pain (>3 mo) in the adipose tissue

Associated conditions

Associated conditions include sleep disturbances; slight-to-moderate dryness of the eyes and the mouth, with a gritty feeling in the eyes in spite of normal tear production (the criteria for Sjögren syndrome are not completely satisfied); an irritable bowel; coccygodynia; vulvovaginitis; vulvodynia; carpal tunnel syndrome; Tietze syndrome; chondromalacia patellae; thyroid malfunction, mainly hypothyreosis; trochanteritis; localized tendonitis; and onset of fibromyalgia (sometimes).[14, 15]

Mode of inheritance

Dercum disease is believed to be transmitted in an autosomal dominant manner with incomplete penetrance.[16, 17] It is particularly strong in the maternal line; however, most reported cases of adiposis dolorosa appear to be sporadic.[18]

Pathophysiology

The understanding of the pathogenesis and mechanism of Dercum disease (adiposis dolorosa) remains unknown. The origin of the pain is obscure, and the disease is better known as a clinical entity rather than as a physiologic or metabolic process. Fatty deposits are thought to cause nerve compression and result in weakness and pain.

A review of the histopathologic findings of Dercum disease showed no consistent histologic abnormality in the adipose tissue that might distinguish these tumors from common sporadic lipomas.[18] In theory, the sudden appearance of the disease together with the incidence of a slight increase in the number of inflammatory cells in the fat could point toward the disease being, in part, an immune defense reaction.[15, 19] Some authors believe that the sympathetic nervous system may play a role in the origin and development of the pain.

The report of a case of Dercum disease developing in association with the use of high-dose corticosteroids and its resolution upon reducing the dose suggests a causal relationship. Therefore, alterations of fat metabolism induced by corticosteroid excess may play a role in the development of this syndrome.[14] An earlier study suggested that a defect in the synthesis of monounsaturated fatty acids may play a role in its development. Further studies are needed to support this hypothesis and to identify a specific biochemical defect.[20]

Dercum disease has been suggested to be an expression of familial multiple lipomas, which is an autosomal dominant disease characterized by multiple, usually asymptomatic lipomas. This observation was derived by studying the family patterns of two siblings with Dercum disease; findings suggested that the disease segregates in an autosomal dominant fashion with variable phenotypic expressivity, ranging from totally asymptomatic to extremely painful lipomas.[21] However, most cases of the disease are found to be sporadic with no specific genetic mutations.[1]

Mutational analysis excluded the 8344A→G mitochondrial mutation seen in other patients with multiple lipomas.[18, 21] The A→G transition at position 8344 in the tRNAlys gene of mitochondrial DNA has been described in the syndrome myoclonic epilepsy and ragged-red fibers (MERRF). A number of reports described the presence of multiple lipomas resembling those of multiple symmetrical lipomatosis in some members of pedigrees with MERRF harboring the 8344 tRNA mutation.[22]

Gamez et al described an unusual syndrome characterized by maternally inherited multiple symmetrical lipomatosis in a pedigree harboring the 8344 mutation in the tRNAlys gene of mitochondrial DNA.[21] Although the probands in their study harbored this mutation and had sensory polyneuropathy, they lacked the typical neuromuscular manifestations of MERRF.

In 2015, an abnormal lymphatic phenotype was discovered in three patients with the disease compared with four female controls using near-infrared fluorescence (NIRF) lymphatic imaging.[23] The lymphatics in the participants with Dercum disease were intact and dilated but could not readily clear lymph when compared with lymphatics in four control patients. Further NIRF imaging revealed masses of fluorescent tissue within the painful nodules, suggesting a lymphovascular etiology.

Etiology

The cause of this poorly understood disorder is unknown, although multiple candidate theories have been proposed, including nervous system dysfunction, lymphovascular disorder, adipose tissue dysfunction, endocrine dysfunction, mechanical pressure, trauma-induced, and inflammation, among others. No theory has been consistently substantiated.

High-dose corticosteroids were the suspected cause in a reported case.[14]

Another case report documented traumatic injury sustained from a motorcycle accident approximately 5 years prior as the cause of Dercum disease (adiposis dolorosa).[24] In two patients with rheumatoid arthritis treated with tocilizumab, an atypical juxta-articular form of Dercum disease developed. After discontinuation of tocilizumab, there was progressive disappearance of the pain associated with the fatty masses.[25]

Epidemiology

Frequency

Dercum disease (adiposis dolorosa) is rare and the prevalence has not been established.

Sex

Dercum disease is 20 times more common in females who are postmenopausal, obese, or overweight than in other people. It can occur in individuals who are not obese. Sixteen percent are males.

Age

Dercum disease is most commonly seen in persons aged 45-60 years. It may occur in women younger than 45 years. A survey of patients with the disease concluded that 85% of patients developed symptoms before the onset of menopause.[7] Adiposis dolorosa is almost never seen in children, however, a case report of a Dercum disease affecting a prepubescent 8-year-old girl was published in 2018.[3]

Prognosis

The course is of Dercum disease (adiposis dolorosa) chronic and may be progressive and more debilitating over time.[1, 26]

Patient Education

Educating patients about the chronicity of Dercum disease (adiposis dolorosa) and the available limited treatment modalities is important. Proper education about the aggravating and relieving factors should be explained.

Addressing any possible needs of those persons with disabilities is important, preferably with the assistance of an occupational therapist and a social worker. Various aids may be needed in the home and at work.

There are several social networking support groups related to Dercum disease. The Dercum Society website featuring information on Dercum disease for patients was created in 2005 by a patient with the disease.[27] Other organizations supporting this disease include the American Chronic Pain Association and the Fat Disorders Research Society.[28]

 

Presentation

History

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 overweight, but, in a short time, obesity ensues. The pain increases with the subsequent increase in fatty tissue and in connection to menstruation. Pain is also worsened with palpation.[1]

The painful lipomas have been reported to occur in any location.[29] It was previously thought that the head and the neck were excluded, but it is now suspected that retrobulbar fat deposits may cause facial pain in some patients.

The first classification system for the disease was developed in 1900 by Giudiceandrea and was subsequently amended by Roux and Viteat, among others.[11, 30] Most recently, a 2012 review proposed the following classification system[13] :

  • Type I: Generalized diffuse form; generalized, widespread painful adipose in the absence of discreet lipomas

  • Type II: Generalized nodular form; widespread painful adipose with concomitant intense pain in and around multiple discreet lipomas

  • Type III: Localized nodular form; pain in and around multiple discreet lipomas

  • Type IV: Juxta-articular form; discreet deposits of excess fat in specific locations, including at the medial aspect of the knee, the hips, and, rarely, the upper arm

The pain varies from discomfort on palpation to excruciating, paroxysmal spontaneous attacks.[9] 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.

Other symptoms, with variable incidence

Problems with mobility may occur for large masses located on the lower extremities.[31]

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 (arthralgia).

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.

Dizziness, paresthesia, and motor impairment were described in a case of a 52-year-old woman, with the authors suggesting that neurological and neuropsychiatric manifestations are likely a relevant and probably underestimated component to the disease.[32]

Bouts of depression (atypical depression, possibly latent) may occur; this finding is not associated with the onset of the disease. This association, among others, has been challenged given the prevalence of depression among obese individuals. However, a 2012 study of 111 patients with Dercum disease (adiposis dolorosa) compared with obese control subjects revealed a statistically significant greater prevalence of depression among patients with disease.[10]

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, as with one patient who developed sepsis secondary to steatonecrosis.[33] Pain is exacerbated with infections.

Others may include constipation, bloating, muscle aches, and sleep disturbances.[34]

Physical Examination

Clinical examination is key for diagnosis. A characteristic physical examination finding is pain that is out of proportion. Palpation of the nodules reveals exquisitely tender subcutaneous nodules, and the most common areas involved include proximal extremities, trunk, and buttocks.[1] Passive and active movement of joints should not increase or decrease the amount of pain.[2]

Dercum disease (adiposis dolorosa) patients are obese (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.[35]

Hyperalgesia is found in the fatty tissue below the skin on light pressure and touch.

Other findings include acral swelling, bruises, and telangiectasias.

Complications

Dercum disease (adiposis dolorosa) can be debilitating and can lead to incapacitation. Also, because Dercum disease symptoms are nonspecific, unnecessary medical procedures, tests, and operations can result in several complications.

Although rare, septicemia leading to septic shock, following necrosis of a fatty tumor, has been reported.[36]

 

DDx

Diagnostic Considerations

Differential diagnoses include fibromyalgia, panniculitis, endocrine disorders, primary psychiatric disorders, multiple symmetric lipomatosis (Madelung syndrome), multiple familial lipomatosis, Proteus syndrome, and benign adipose tissue tumors.[1]

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, a relationship exists between pain and body weight, with pain in the fatty tissue and obesity being fundamental criteria for the diagnosis. In addition, the pain is often more general and more severe than in fibromyalgia.

Panniculitis presents with painful inflammatory nodules of the subcutaneous fat; however, the findings of erythema, ulceration, and atrophic scarring, as well as history and location, differentiate it from Dercum disease. Biopsy is diagnostic.[1]

Cushing syndrome and hypothyroidism are endocrine disorders also in the differential. These two diseases present with weight gain and generalized pain that is not localized to subcutaneous tissue as is seen in Dercum disease.[1]

Depression can also present similarly to Dercum disease as chronic pain conditions are often associated with depression.[1]

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.[37]

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 by their lack of disabling pain.[37]

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.

A patient with increasingly painful nodules resembling adiposis dolorosa was found to have calciphylaxis, and it is noted that the diseases may present similarly.[38]

Other conditions to consider include MERRF syndrome with lipomatous lesions, neurofibromatosis type 1, and multiple endocrine neoplasia type 1.[28]

Differential Diagnoses

 

Workup

Laboratory Studies

Results of hormonal studies to rule out Cushing syndrome, thyroid abnormalities, and other endocrinologic abnormalities are characteristically normal.

Dercum disease (adiposis dolorosa) patients might have associated slight-to-moderate elevation of cholesterol levels.

Erythrocyte sedimentation rate results may be slightly elevated.

Coagulation test results usually are normal.

In spite of obesity, hypertension and type 2 diabetes mellitus are often absent.

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.[14, 15]

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.[37, 39]

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.[40] 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 MRI may aid in the diagnosis of Dercum disease (adiposis dolorosa).[35, 41, 42]

On MRI, the lesions appear oblong, and this may be due to septal distortion that is seen on histopathological evaluation. In a study by Tins et al of 13 patients with Dercum disease, lesions of the condition were found to be markedly hyperechoic on ultrasonography, superficial in location, and distinct from characteristic lipomas.[43] Further, when validated on more than 6,000 MRIs, they appeared as ill-defined, nodular, “blush-like” subcutaneous fat on unenhanced MRI with a decreased T1-weighted signal. No case of Dercum disease was without these features in the study, and the authors concluded that these findings, along with multiple subcutaneous fatty lesions, is “very suggestive and possibly pathognomonic” for the condition.

Histologic Findings

A review of histopathologic findings did not reveal any significant features that might distinguish Dercum disease (adiposis dolorosa) tumors from common sporadic lipomas. An inflammatory response with lymphocytes and macrophages is seen on biopsy, although the inflammation may not be any more pronounced than that of a healthy patient with obesity without a diagnosis of Dercum disease.[1] 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.

Other Tests

Bioimpedence has been described as a noninvasive tool that may help to distinguish Dercum disease (adiposis dolorosa) from lipedema. Regional bioimpedence measures (tissue water bioimpedence in the upper and lower extremities) seem to differ between Dercum disease and lipedema.[44]

 

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]

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]

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.

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]

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.

 

Diet

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

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.

 

Medication

Medication Summary

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

Corticosteroids

Class Summary

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)

Prednisone may provide pain relief. Caution should be used because the adverse effects may outweigh the benefits. Prednisone is an immunosuppressant for treatment of autoimmune disorders; it may decrease inflammation by reversing increased capillary permeability and suppressing PMN activity. Prednisone stabilizes lysosomal membranes and suppresses lymphocyte and antibody production.

Anesthetics

Class Summary

Lidocaine is reported to provide pain relief for 10 hours to several months. 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 anesthetic (Dilocaine, Xylocaine)

Lidocaine anesthetic decreases permeability to sodium ions in neuronal membranes. This results in inhibition of depolarization, blocking transmission of nerve impulses.

Analgesics

Class Summary

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

Propoxyphene products were withdrawn from the United States market on November 19, 2010. The withdrawal was based on new data showing QT prolongation at therapeutic doses. For more information, see the FDA MedWatch safety information.

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

Acetaminophen is the 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.