Dercum Disease 

Rare Diseases / By

Dercum Disease – Introduction

  • Dercum disease (also called adiposis dolorosa) is a rare condition characterized by overweight or obesity and chronically painful subcutaneous adipose tissue(1,2)
  • Dercum disease is reported to present primarily in adults aged 30-50 years and more commonly occurs in women(1,2)

Types

  • Dercum disease may be classified into 1 of 4 types – generalized diffuse, generalized nodular, localized nodular, and juxta-articular(1,2)
    • generalized diffuse type is characterized by widespread painful adipose tissue without distinct lipomas
      • small adipose deposits may be palpable
      • pain occurs throughout the body, including areas without detectable lumps
    • generalized nodular type is characterized by multiple lipomas and generalized pain
      • pain may occur in adipose tissue not affected by lipomas
      • intense pain may also occur within and around lipomas
    • localized nodular type is characterized by multiple lipomas with pain localized to lipomas and immediate surrounding area (no generalized pain)
    • juxta-articular type is characterized by solitary deposits of excess fat near large joints, such as the knee, hip, or elbow

Also Called

  • adiposa dolorosa or adiposis dolorosa
  • adiposalgia
  • adipose tissue rheumatism
  • Anders disease or Anders syndrome
  • Dercum’s disease
  • Dercum syndrome or Dercum’s syndrome
  • fatty tissue rheumatism
  • lipalgia
  • lipomatosis dolorosa
  • Morbus Dercum
  • neurolipomatosis

Epidemiology

Who Is Most Affected

  • Dercum disease most commonly presents in adults aged 35-50 years, and is reported to be 5-30 times more common in women than in men(1,2)

Incidence/Prevalence

  • rare condition(1,2)
  • incidence and prevalence are unknown; there are no studies available reporting incidence or prevalence(1,2)

Associated Conditions

  • obesity(1,2)
  • depression(1,2)
  • anxiety(1,2)
  • sleep disturbances(1,2)

Etiology and Pathogenesis

  • etiology and pathogenesis of Dercum disease are unknown(1,2)
    • reported hypotheses include endocrine, nervous system, adipose tissue, or lymphovascular dysfunction, although there is minimal evidence of a role for these factors
    •  inflammation may be involved, although inflammatory markers such as erythrocyte sedimentation rate and C-reactive protein are generally normal in patients with Dercum disease, and inflammation found on histology of fat biopsies in patients with Dercum disease does not appear more severe than seen in individuals with obesity without Dercum disease (J Inflamm (Lond) 2011 Sep 28;8(1):24)
    •  trauma has been reported as a cause of localized Dercum disease
  • autosomal dominant inheritance with incomplete penetrance may play a role(1,2)
    • some familial cases have been reported
    • most patients with Dercum disease do not appear to have family history of the condition
    • genetic studies have not identified a specific mutation
  • Dercum disease after histoplasmosis, coccidioidomycosis, or Lyme disease reported in 7 women (aged 24-58 years) in case series (IDCases 2020;19:e00682full-text)
  • trauma to the upper extremities caused by a motor vehicle accident reported to induce localized Dercum disease in 39-year-old man in case report (BMJ Case Rep 2018 Mar 28;2018:doi:10.1136/bcr-2017-223869full-text)

History and Physical

Clinical Presentation

  • Dercum disease most commonly presents in middle-aged women(1,2)
  • primary symptoms of Dercum disease are overweight or obesity and chronically painful adipose tissue(1,2)
    • pain is typically burning or aching, severe, and chronic (> 3 months)
    • can range in severity from hyperalgesia in subcutaneous fatty tissue and discomfort with palpation to spontaneous, paroxysmal pain attacks
    • pain may be generalized throughout the body or localized to the area of adiposity and lipomas
    • lipomas or small, subcutaneous adipose lumps may be palpable and painful
  • when present, lipomas or small, subcutaneous adipose lumps may be palpable and painful, and most commonly occur in the buttocks, thighs, upper arms, and abdomen(1,2)
  • common symptoms include(2)
    • fatigue or weakness (asthenia)
    • tachycardia
    • dyspnea
    • constipation
    • joint and/or muscle pain
    • easy bruising
    • bloating
  • associated psychiatric and neurological manifestations may include(1,2)
    • depression
    • anxiety
    • cognitive impairment
    • dementia
    • epilepsy
    • impaired memory
    • difficulty concentrating
    • emotional instability
    • sleep disturbances
  • patients may also present with diabetes(1)
  • rare signs and symptoms may include(1,2)
    • mastalgia
    • necrosis
    • sepsis
    • dysarthria
    • visual pursuit defects
    • progressive dystonia
    • hydronephrosis
  • women with Dercum disease may be more likely to have depression than otherwise healthy women with obesity
    •  based on case-control study
    • 152 women with pain due to generalized diffuse Dercum disease (no lipomas) or without Dercum disease or pain who were having planned abdominoplasty were evaluated with Montgomery Åsberg Depression Rating Scale (MADRS) (query on suicidal ideation omitted)
      • Dercum disease in 111 women (mean age 53 years, mean body mass index [BMI] 34 kg/m2)
      • no Dercum disease or pain in 41 women having planned abdominoplasty (mean age 50 years, mean BMI 34 kg/m2)
    • MADRS includes 9 scales: mood, feelings of unease, sleep, appetite, concentration, initiative, emotional involvement, pessimism, and zest for life
      • each scale scored 0-6 with higher scores indicating more severe difficulties
      • total score 0-54 points with score of 0-12 indicating no depression, 13-19 indicating light depression, 20-34 indicating moderate depression, and ≥ 35 indicating severe depression
    • comparing women with Dercum disease vs. women without Dercum disease
      • mean MADRS total score 14 vs. 4 (p = 0.014)
      • mean MADRS mood score 1 vs. 0 (p = 0.018)
      • mean MADRS pessimism score 1 vs. 0 (p = 0.022)
      • mean MADRS zest for life score 1 vs. 0 (p = 0.009)
      • no depression in 44% vs. 85% (no p value reported)
      • light depression in 30% vs. 15% (no p value reported)
      • moderate depression in 25% vs. 0% (no p value reported)
      • severe depression in 1% vs. 0% (no p value reported)
    • no significant differences between groups in feelings of unease, sleep, appetite, concentration, initiative, or emotional involvement
    • Reference – BMC Psychiatry 2012 Jul 3;12:74full-text

Physical

  • small, soft adipose deposits or lipomas may be palpable and tender to palpation(1,2)
  • lipomas may vary in size and firmness and are typically found in the legs, arms, and anterior or posterior trunk
    • buttocks lipomas reported in about 70% of patients
    • scalp and neck lipomas reported in about 33% of patients
    •  facial lipomas reported in about 20% of patients
  • painful adipose tissue (with or without lipomas) is also commonly located in(1,2)
    • subcostal area
    • medial region of upper arms
    • lateral and medial regions of the thighs
  • skin over painful area is typically unchanged, without altered skin pigmentation or cutaneous breakage(1,2)

Diagnosis

Making the Diagnosis

  • diagnosis of Dercum disease is based on (1,2)
    • characteristic signs and symptoms typically seen in postmenopausal women of multiple chronically painful, fatty masses, generalized obesity and asthenia with or without neuropsychiatric disturbances
    • exclusion of other similar and more common conditions, such as fibromyalgia, Cushing syndrome, lipedema, and panniculitis
  • Dercum disease may be classified into 1 of 4 types – generalized diffuse, generalized nodular, localized nodular and juxta-articular(1,2)
    • generalized diffuse type is characterized by widespread painful adipose tissue without distinct lipomas
      • pain occurs throughout the body
      • small adipose deposits may be palpable, but pain is not limited to the deposits and may occur throughout the body
    • generalized nodular is characterized by multiple lipomas and generalized pain
      • pain may occur in adipose tissue not affected by lipomas
      • intense pain may also occur within and around lipomas
    • localized nodular is characterized by multiple lipomas with pain localized to lipomas and immediate surrounding area only (no generalized pain)
    • juxta-articular type is characterized by solitary deposits of excess fat near large joints, such as the knee, hip, or elbow

Differential Diagnosis

  • for generalized forms of Dercum disease, rule out(1,2)
    • lipedema
      • usually limited to lower extremities
      • characterized by bilateral symmetric occurrence and homogeneous fat deposition
      • typically occurs in women and develops after puberty
      • S1 guidelines for lipedema can be found in J Dtsch Dermatol Ges 2017 Jul;15(7):758
    • panniculitis
      • associated with inflammation and painful subcutaneous masses with or without vasculitis
      • septal panniculitis without vasculitis is characterized by erythematous nodules that typically spontaneously resolve without scarring within 6 weeks
      • lobular panniculitis with vasculitis is characterized by erythematous nodules that are recurrent and tender, frequently ulcerate, and heal with atrophic scars
      • commonly occurs in the lower extremities
    • lymphedema
      • caused by accumulation of protein-rich interstitial fluid in the skin
      • can be managed with compression or lymphatic massage
    • fibromyalgia
      • characterized by widespread muscle pain and painful response to pressure on specific sites
      • associated with sleep disturbances, cognitive issues, depressive symptoms, and fatigue
      • does not present with painful lipomas
    • endocrine disorders such as Cushing syndrome and hypothyroidism, which are not typically characterized by painful adipose tissue
    • Proteus syndrome
    • Weber-Christian disease
    • Fröhlich’s syndrome
    • progressive lipodystrophy
  • for localized forms, rule out(1,2)
    • multiple symmetric lipomatosis (Madelung’s syndrome, Lanois-Bensaude syndrome)
      • characterized by excessive fat deposition in the head, neck, and upper torso, but adipose deposits are not typically painful
      • usually occurs in men
      • often associated with alcoholism
    • adipose tissue tumors
    • familial multiple lipomatosis
    • neurofibromatosis type I
    • adenolipomatosis
    • multiple endocrine neoplasia type 1 (MEN1), which can be characterized by multiple subcutaneous lipomas that are not painful
    • myoclonic epilepsy with red ragged fibers (MERRF), which can be characterized by multiple lipomas that are not painful
    •  if significant breast pain, consider other causes of mastalgia
  • abdominal pain, shortness of breath, fibromyalgia, diabetes, lipomas, and migraines may be more common and easy bruising and visible veins on legs may be less common in patients with Dercum disease compared with patients with lipedema
    •  based on retrospective cohort study
    • 272 patients with Dercum disease and/or lipedema were included
      • lipedema in 58.8% (mean age 50 years, 99.4% female)
      • Dercum disease in 34.6% (mean age 49 years, 84% female)
      • both Dercum disease and lipedema in 6.6% (mean age 50 years, 100% female)
    • comparing Dercum disease vs. lipedema
      • symptoms
        • abdominal pain in 27% vs. 16.8% (p = 0.04)
        • easy bruising in 15% vs. 28.1% (p = 0.02)
        • shortness of breath in 27.7% vs. 12.5% (p = 0.002)
        • visible veins on legs in 13.8% vs. 25% (p = 0.02)
      • comorbidities
        • fibromyalgia in 28% vs. 10% (p = 0.0003)
        • diabetes in 16% vs. 6% (p = 0.007)
        • lipomas in 20% vs. 0% (p < 0.0001)
        • migraines in 21% vs. 7% (p = 0.005)
    • no significant differences between patients with lipedema vs. patients with Dercum disease in
      • symptoms (anxiety, constipation, depression, diarrhea, difficulty sleeping, fatigue, headaches, itching, joint aches, low back pain, muscle aches, muscle weakness, nocturia, poor concentration, swelling, or unexplained weight gain)
      • comorbidities (arthritis, depression, hypertension, or hypothyroidism)
    • Reference –Int J Obes (Lond) 2017 Feb;41(2):240

Testing Overview

  • no specific testing is useful for diagnosing Dercum disease(1,2)
  • routine laboratory testing that may help rule out other possible causes of signs or symptoms includes
    • complete blood count
    • erythrocyte sedimentation rate (ESR)
    • C-reactive protein (CRP)
    • creatine kinase
    • metabolic panel
    • thyroid function testing
  • lesions appear in superficial subcutaneous fat on ultrasound and magnetic resonance imaging (MRI)(1)
    • most lesions are < 2 cm in diameter and oblong with long axis parallel to skin
    • ultrasound shows hyperechoic lesions without surrounding edema and with no demonstrable flow on Doppler imaging
    • MRI may show blush-like appearance with decreased T1-weighted signal and increased signal on short tau inversion recovery (STIR) imaging
    • imaging may reveal more lipomas and adipose deposits than clinically detected
    • no abnormalities seen in deep fat
  • biopsy of affected fat or lipoma generally shows fat masses without signs of inflammation(1,2)

Management

Management Overview

  • the goal of management for Dercum disease is to reduce pain; however, evidence of efficacy of management approaches is limited and based on observational studies
  • options for treatment
    • medications such as nonsteroidal anti-inflammatory drugs, calcium channel modulators such as pregabalin or oxcarbazepine and lidocaine are commonly advised for pain relief
    • intralesional deoxycholic acid (Kybella) injection to lipoma reported to reduce pain of lipoma (level 3 [lacking direct] evidence)
    • surgery and procedures
      • “dry” liposuction may reduce pain in women with generalized diffuse Dercum disease (level 2 [mid-level] evidence)
      • dermolipectomy reported to reduce pain of painful lipomas (level 3 [lacking direct] evidence)
    • other management
      • hypobaric pneumatic compression reported to reduce pain severity and weight in 10 adults with Dercum disease (level 3 [lacking direct] evidence)
      • subcutaneous adipose tissue (SAT) therapy reported to reduce fat mass but not pain in 7 women with Dercum disease (level 3 [lacking direct] evidence)
      • transcutaneous frequency-modulated electromagnetic neural stimulation (FREMS) reported to improve pain and function in patients with Dercum disease (level 3 [lacking direct] evidence)
      • subcutaneous deoxycholic acid reported to reduce pain and lipoma volume in adults with Dercum disease (level 3 [lacking direct] evidence)
  • effect of weight loss on Dercum disease is unclear and evidence to support it is inconsistent
    • patients with Dercum disease have reported that diet and exercise have not been effective for weight loss(2)
    • fatty deposits may not be affected by weight loss(2)
    • failure to lose > 15.4 lbs (7 kg) of weight despite laparoscopic roux-en-Y gastric bypass surgery reported in 45-year-old woman with Dercum disease in case report (Surg Obes Relat Dis 2011 Mar;7(2):243)
    • d-amphetamine reported to result in weight loss, reduced pain, and reduced lipid deposition in the liver in 2 adults with Dercum disease (level 3 [lacking direct] evidence)

Medications

  • evidence for use of medications for management of pain associated with Dercum disease is limited to case series and case reports (Fed Pract 2019 Nov;36(11):529full-text)
  • medication options include
    • nonsteroidal anti-inflammatory drugs (NSAIDs)
      • reported to have limited efficacy(1)
      • NSAIDs reported to improve pain in 88.8% of 89 adults in cross-sectional study of 110 adults (mean age 48 years, 82.7% female, 80.9% non-Hispanic White) with Dercum disease (The Endocrinologist 2007 Dec;17(6):326)
    • calcium channel modulators
      • oxcarbazepine or pregabalin have been used for patients with Dercum disease (Fed Pract 2019 Nov;36(11):529full-text)
      • successful management of Dercum disease with pregabalin 75 mg twice daily for 1 week increasing to 150 mg 3-4 times daily in second week and manual lymphatic massage for 60 minutes twice weekly with follow-up for 8 weeks reported in 58-year-old woman in case report (Rheumatol Int 2008 Nov;29(1):17)
    • lidocaine(1,2)
      • can be administered as intralesional injection, transdermal application, or IV infusion
      • mechanism of action for management of Dercum disease is unclear, but lidocaine is believed to block sympathetic activity and impulse conduction in peripheral nerves
      • lidocaine 5% patch or topical lidocaine or lidocaine/prilocaine reported as potentially helpful
  • evidence for other medications in case reports
    • successful management of Dercum disease with ketamine 500 mg IV every 14 weeks plus acupuncture once monthly reported in 53-year-old man in case report (Fed Pract 2019 Nov;36(11):529full-text)
    • intralesional deoxycholic acid (Kybella) injection to lipoma reported to reduce pain and self-reported but not clinician-reported lipoma size at 3 months’ follow-up in 46-year-old man with Dercum disease in case report (Dermatol Surg 2019 Dec;45(12):1718)
    • d-amphetamine reported to result in decreased weight, pain, and lipid deposition in the liver in 2 adults with Dercum disease (Am J Med 2018 Jun;131(6):705)
    • metformin 850 mg 3 times daily for treatment of type 2 diabetes reported to improve pain with 1 year follow-up in 48-year-old man with Dercum disease in case report (Pain Med 2012 Nov;13(11):1526)
    • infliximab 3 mg/kg IV every 6 weeks plus methotrexate 20 mg/week for treatment of ankylosing spondylitis reported to improve symptoms of comorbid Dercum disease in 50-year-old woman in case report (J Eur Acad Dermatol Venereol 2007 May;21(5):717)
  • antidepressants may be suggested but evidence for their use in the management of Dercum disease is lacking(1)

Surgery and Procedures

Liposuction

  • liposuction can be completed with “dry” (no local anesthetic or epinephrine) or tumescence technique(1)
  • mechanism of pain reduction associated with liposuction remains unclear, but is hypothesized to partly involve the destruction of nerve plexuses in adipose tissue(1,2)
  • pain relief may decrease over time and pain may recur(1,2)
  • “dry” liposuction may be associated with reduced pain in women with generalized diffuse Dercum disease (level 2 [mid-level] evidence)
    •  based on prospective cohort study
    • 152 women with pain due to generalized diffuse Dercum disease (no lipomas) or without Dercum disease or pain who were having planned abdominoplasty were evaluated
      • liposuction in 53 women (mean age 52 years, mean body mass index [BMI] 34 kg/m2) with Dercum disease
      • no liposuction in 58 women (mean age 51 years, mean BMI 35 kg/m2) with Dercum disease
      • abdominoplasty in 41 women (mean age 49 years, mean BMI 38 kg/m2) without Dercum disease or pain
    • liposuction done with “dry technique” under general anesthesia, epidural, or spinal block and with anticoagulation (most commonly dextran) during surgery
    • postoperative compression with bandages or corset maintained for 6 weeks
    • subjective quality and intensity of pain measured with visual analog scale (VAS) and number of words chosen; objective pain response measured by algometer applying mechanical pressure at Newton/cm2 and measuring pain pressure thresholds (PPT)
    • comparing liposuction vs. no liposuction in patients with Dercum disease
      • mean VAS pain score
        • at 3 months 3.4 vs. 6.2 (p < 0.001)
        • at 1 year 4.4 vs. 5.2 (p = 0.074)
        • at 5 years 4.9 vs. 6.1 (p = 0.028)
      • mean abdominal PPT
        • at 3 months 23.1 vs. 16.3 (p < 0.001)
        • at 1 year 22.9 vs. 16.5 (p < 0.001)
        • at 5 years 23.4 vs. 17.7 (p < 0.001)
    • Reference – Pain Med 2011 Jun;12(6):942full-text
    • compared to not having liposuction, liposuction associated with significant improvement in psychological general well-being at 3 months follow-up, but not at 1- or 2-years follow-up in patients with Dercum disease (J Plast Surg Hand Surg 2012 Sep;46(3-4):252)

Dermolipectomy

  • dermolipectomy involves excision and removal of excess adipose tissue followed by drainage (Wien Med Wochenschr 2015 Sep;165(17-18):374)
  • dermolipectomy appears to reduce pain in patients with Dercum disease, but evidence is limited to case reports and case series
    • dermolipectomy of adipose tissue reported to relieve pain in 4 women with juxta-articular Dercum disease (level 3 [lacking direct] evidence)
      •  based on case series
      • 4 women aged 52-83 years with juxta-articular Dercum disease of the knee for > 10 years had dermolipectomy
      • dermolipectomy was performed under general anesthesia and consisted of a fish-shaped excision and removal of excess adipose tissue down to the muscle fascia followed by cross-perforated drainage for > 24 hours
      • length of hospital stay ranged from 3 to 5 days
      • pain-free outcome reported by all women within 1-5 weeks after surgery
      • postoperative lymph fistula in 2 women
      • Reference –Wien Med Wochenschr 2015 Sep;165(17-18):374
    • minimal incision lipectomy via a 4 mm punch biopsy site reported to reduce pain and lipomas at 6 months’ follow-up in 46-year-old woman with localized nodular Dercum disease and diabetes mellitus type 2 in case report (Dermatol Ther 2020 Jun 3;e13751)
    • dermolipectomy of a large exophytic adiposis mass of the medial thigh with vacuum-assisted wound closure followed by delayed split-thickness skin graft reported to improve function and pain at 7 weeks’ follow-up in 77-year-old woman in case report (Cureus 2020 Mar 15;12(3):e7282full-text)
    • improved pain and no recurrence at 7 months’ follow-up after surgical removal of painful lipomas reported in 76-year-old woman in case report (An Bras Dermatol 2019 Mar;94(2):251full-text)

Other Management

  • additional management may include therapy and patient education, but evidence for their use in the management of Dercum disease is lacking(1)
  • electrical stimulation
    • frequency rhythmic electrical modulation system (FREMS) reported to reduce pain and improve functioning in adults with type II generalized nodular form of Dercum disease (level 3 [lacking direct] evidence)
      •  based on uncontrolled trial with changes to analgesia medication over trial
      • 9 adults with type II generalized nodular form of Dercum disease were assigned to FREMS therapy for 5 cycles at 3-month intervals over 1 year
        • FREMS was transcutaneous electrical simulation given over 30 minutes with sequences of biphasic, asymmetric pulses (1-50 hertz) consisting of active phase of high negative voltage spike of extra short duration and recharging phase of low voltage activity of longer duration via electrodes placed over most painful areas
        • patients set own maximal electrical stimulation threshold (up to 300 volts) by progressively increasing voltage through handheld remote control
      • outcomes
        • pain assessed using visual analog scale (VAS) score at 1 year (range 0-100 points, with 100 indicating worst pain
        • health status assessed using Short Form 36 (SF-36) questionnaire (range 0-100 points, with higher score indicating better functioning)
      • 5 patients completed all cycles and 7 patients (median age 49 years, 100% female) were included in analysis
      • median scores comparing baseline vs. 12 months
        • VAS pain score 92 points vs. 52.5 points (p = 0.0597)
        • SF-36 body pain score 22 points vs. 55 points (p = 0.0015)
        • SF-36 physical functioning score 40 points vs. 70 points (p = 0.047)
        • SF-36 role limitation due to physical impairment score 0 points vs. 50 points (p = 0.0128)
        • SF-36 social functioning score 25 points vs. 56 points (p = 0.021)
        • SF-36 vitality score 15 points vs. 30 points (p = 0.0439)
      • no significant differences in SF-36 general health, role limitation due to emotional problems, or mental health scores at 12 months compared to baseline
      • no major adverse events were reported
      • Reference – Medicine (Baltimore) 2021 Dec 23;100(51):e28360full-text
    • transcutaneous frequency-modulated electromagnetic neural stimulation (FREMS) for 4 cycles of 10 sessions each over 6 months reported to improve pain and function in 57-year-old male patient with generalized Dercum disease in case report (Medicine (Baltimore) 2015 Jun;94(24):e950full-text)
  • hypobaric pneumatic compression reported to reduce pain severity and weight in adults with Dercum disease (level 3 [lacking direct] evidence)
    •  based on uncontrolled trial
    • 10 adults (mean age 48 years, 60% female, 90% non-Hispanic White) with Dercum disease were treated with whole-body cyclic hypobaric pneumatic compression in a high-performance altitude simulator simulating altitudes up to 3,200 meters for 20 minutes once or twice daily for 5 consecutive days (mean 9.4 sessions completed)
    • at baseline, mean weight 195.6 lbs (88.7 kg) and mean body mass index (BMI) 28.3 kg/m2
    • comorbid fibromyalgia in 5 adults
    • compared to baseline, significant reduction in pain severity and mean weight on day 5 (p < 0.05)
    • no significant difference from baseline in pain quality
    • Reference – J Pain Res 2010 Aug 20;3:147full-text
  • subcutaneous adipose tissue (SAT) therapy reported to reduce fat mass but not pain in women with Dercum disease (level 3 [lacking direct] evidence)
    •  based on uncontrolled trial
    • 7 patients (mean age 46 years, 100% female, mean BMI 32.1) with lipedema, Dercum disease, or both had SAT therapy 3 times weekly for 4 weeks
      • lipedema only in 3 women
      • lipedema and diffuse Dercum disease in 3 women
      • diffuse Dercum disease only in 1 woman
    • SAT therapy consisted of deep manual massage of fat, muscle, and fascia for 90 minutes, including 10 minutes each on the neck, upper back, lower back, back upper legs, back lower legs, front upper legs, front lower legs, abdomen, arms, and chest, and 5 minutes each on the feet and head/face
    • follow-up at 4.5 months
    • total body mass and leg fat mass significantly decreased from baseline (p < 0.05)
    • no significant decrease from baseline in pain by visual analogue scale or arm fat mass
    • Reference – Clin Obes 2018 Dec;8(6):398
  • subcutaneous deoxycholic acid reported to reduce pain and lipoma volume in adults with Dercum disease (level 3 [lacking direct] evidence)
    •  based on small nonrandomized trial without statistical comparisons
    • 3 female adults with multiple painful subcutaneous nodules and ultrasound imaging consistent with Dercum disease had their most painful lipomas treated with 2 mL deoxycholic acid 10 mg/mL subcutaneously vs. no treatment (control)
    • lesions were measured by blinded outcome assessors
    • patient 1 (1 treated and 1 not treated lipoma)
      • at 4 months after treatment, 34.6% reduction in lipoma volume with deoxycholic acid vs. 111% increase in lipoma volume with no treatment
      • patient reported decreased pain in treated area over 1-year follow-up
    • patient 2 at 6 months after treatment (2 treated and 1 not treated lipomas)
      • 41%-68% reduction in lipoma volume with deoxycholic acid
      • 22% increase in lipoma volume with no treatment
    • patient 3 at 2 months after treatment (1 treated and 1 not treated lipoma)
      • 30.3% reduction in lipoma volume with deoxycholic acid vs. 15% increase in lipoma volume with no treatment
      • patient reported no pain or other adverse event
    • Reference – Cutis 2023 Apr;111(4):E4

Complications

  • Dercum disease may lead to impairments in functioning and mobility(1,2)
  • steatocutaneous necrosis in a lipoma and subsequent septic shock reported in 63-year-old woman with Dercum disease (Ann Chir Plast Esthet 2005 Jun;50(3):247)

Prognosis

  • Dercum disease appears to be a chronic condition with pain that may increase or remain constant over time(1,2)

Prevention and Screening

  • not applicable

Guidelines and Resources

Guidelines

Review Articles

  • to search MEDLINE for (Dercum Disease) with targeted search (Clinical Queries), click therapy, diagnosis, or prognosis

Patient Information

References

  1. Kucharz EJ, Kopeć-Mędrek M, Kramza J, Chrzanowska M, Kotyla P. Dercum’s disease (adiposis dolorosa): a review of clinical presentation and management. Reumatologia. 2019;57(5):281-287full-text.
  2. Hansson E, Svensson H, Brorson H. Review of Dercum’s disease and proposal of diagnostic criteria, diagnostic methods, classification and management. Orphanet J Rare Dis. 2012 Apr 30;7:23full-text.

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