Mixed generalized/juxta-articular form of Dercum’s disease

Dercum’s disease is a rare disorder with subcutaneous formation of fatty tissue (lipomas) with symptoms of pain, fatigue, stiffness, weakness and in some cases arthritis.

Differential diagnosis excluded fibromyalgia, benign symmetric lipomatosis, lipedema, adenolipomatosis, and panniculitis.

Dercum’s disease appears in nodular, generalized and juxta-articular forms. In this short report, we report a patient with mixed generalized/juxta-articular form. The patient responded well to medication with paracetamol administered in low doses. We recommended continuation of this treatment as well as monitoring for diabetes or glucose intolerance. Metabolic disturbances are common in patients with Dercum’s disease.




Lipoma Treatment Review

The following link provides a review of lipoma excision techniques and possible alternative technologies that have been tested or are being developed. Of the reviewed methods, liposuction is the most effective overall in removing adipose tissue, but is hindered by associated costs. Deeper lipomas may be ablated with laser technologies, although larger incisions are required. Additionally, study results of ultrasound and pharmaceutical methods are promising, but require FDA approval before becoming a prevalent practice.
Conclusion: Although lipoma affects a large portion of the population, the majority of removal procedures for subcortical lipomas are still performed with a standard set of scalpels and scissors. Unfortunately, the standard methods can result in significant scarring. The most effective adipose tissue removal method that is FDA approved is liposuction, but the required equipment and training are cost prohibitive for many surgeons. New methods are desired to effectively remove an entire lipoma with reduced scarring and costs. Ultrasound and pharmaceutical methods have shown promise, but still require FDA approval. Laser technologies have been effective for deeper lipomas, but require very large incisions. Our society and the industry would benefit from the development of a novel, directed approach to lipoma excision, which may utilize a combination of components from the many emerging technologies.


Lipedema: diagnostic and management challenges

Lipedema is an uncommon disorder characterized by localized adiposity of the lower extremities, often occurring in females with a family history of the condition. The adiposity extends from hips to ankles and is typically unresponsive to weight loss.

It is important to distinguish Dercum’s disease (adiposis dolorosa) from lipedema, which is characterized by multiple painful lipomatous tumors, differing from the diffuse soft fatty deposition seen with lipedema.

Full text w/photos: https://www.dovepress.com/lipedema-diagnostic-and-management-challenges-peer-reviewed-fulltext-article-IJWH


Dercum’s disease is a rare disorder described by generalized obesity with painful adipose tissue. The clinical symptoms presented were multiple painful fatty masses, fatiguablity, swelling of fingers, morning stiffness, cognitive dysfunction, headache, anxiety, rapid heartbeat, shortness of breath, bloating, constipation, easy bruisability, joint aches, muscle aches, mood swing, delirium and dementia. Dercum’s disease affects women more frequently than men. Elevated erythrocyte sedimentation rate, alpha-1 antitrypsin, orosomucoid, haptoglobin, compliment factors C3.C4, Clq and Cls have been found in Dercum’s disease. Differential diagnosis includes Fibromyalgia, Madelung’s, Familial multiple lipomatosis, Proteus syndrome, Weber-christian disease, Neurofibromatosis type1, Frohlich syndrome, Lipodystrophia and Metabolic disorders. The diagnosis is made clearly when the differential diagnoses have been excluded. The main goal of treatment in Dercum’s disease includes the pain reduction with surgical interventions (liposuction, excision), pharmacological therapies (analgesics, membrane stabilizing agents, corticosteroids, calcium channel modulators, methotrexate and infliximab, Interferon α-2b) and other alternative such as Rapid cycling hypobaric pressure and Frequency Modulated Electromagnetic Neural Stimulation. We propose a review on definition, classification, pathophysiology, diagnostic methods and treatment.

Full Text: http://www.ejmanager.com/mnstemps/36/36-1470739577.pdf


Rapid Weight Gain

Rapid Weight Gain: A Common Presentation of Adipose Tissue Disorders

Weight gain has long been assumed to be a consequence of impaired energy balance due to poor feeding habits and low physical activity. We examined 40 patients who were referred for rapid weight gain or difficulty losing weight without obvious endocrine or non-endocrine causes. Most of the subjects also complained of fatigue and chronic pain that localized to the subcutaneous fat tissue. We examined our subjects systematically for subcutaneous adipose tissue accumulation in the upper back, deltoid regions, upper arms, trunk, abdomen, hips, buttocks and thighs. We also palpated for lipomas and fat nodules, and assessed abnormal or exaggerated fat distribution in unusual locations. Discrete lipomas in the abdomen, hips, buttocks and upper arms were frequenlty found in patients with rapid weight gain. More than half of our subjects have a painful adipose tissue disorder, adiposis dolorosa, (Dercum’s disease), mostly the nodular subtype. Three subjects had bilateral mastectomy because of mastalgia, 2 subjects have sciatic pain from lipoma growth in the spine, and 2 subjects had been hospitalized for panniculitis. The majority of the subjects have concurrent lipoedema, causing hip and thigh pain. History of previous surgery to remove lipomas is common, and tissue removed surgically in 3 subjects revealed angiolipomas. Other subjects who present with rapid weight gain have non-painful multiple symmetric lipomatosis, familial multiple lipomas, or generalized obesity. In conclusion, many subjects who present for rapid weight gain have abnormal lipomatous growth, which needs to be distinguished from generalized obesity. Dercum’s disease, or painful adipose tissue disorder, is a generally unrecognized condition that presents as rapid weight gain usually with chronic pain. The etiology of adiposis dolorosa remains to be determined.
Nothing to Disclose: A. A. Jamalallail, M.B.B.S., A. U. Rehman, A. Kaur, MBBS, B. C. Villafuerte, MD.
– See more at: http://press.endocrine.org/doi/abs/10.1210/endo-meetings.2016.OABA.8.FRI-644#sthash.oEbUXfe5.dpuf

Case Study: Temporal Adiposis Dolorosa

The clinical and imaging features are consistent with focal adiposis dolorosa likely associated with long-term glucocorticoid use. Adiposis dolorosa (Dercum’s disease) refers to painful fatty tissue in an overweight or obese individual. The epidemiology, etiology and pathogenesis of adiposis dolorosa are not clear but long-term glucocorticoid use for rheumatologic diseases has been associated with focal adiposis dolorosa.

Full Text & Images: http://journals.aace.com/doi/pdf/10.4158/EP161236.VV

ENDOCRINE PRACTICE Rapid Electronic Article in Press Rapid Electronic Articles in Press are preprinted manuscripts that have been reviewed and accepted for publication, but have yet to be edited, typeset and finalized. This version of the manuscript will be replaced with the final, published version after it has been published in the print edition of the journal. The final, published version may differ from this proof.

Juxta-articular adiposis dolorosa (Dercum’s disease type IV): report of four cases and treatment by dermolipectomy

Juxta-articular adiposis dolorosa is a rare subtype of Dercum’s disease. It manifests mainly on the medial parts of the knees. Pain and impaired mobility are common symptoms. We report on four females (aged between 52 and 83 years) who suffered from juxta-articular adiposis dolorosa for more than 10 years. These patients were successfully treated by dermolipectomy resulting in dramatically improved pain and mobility. Adverse effects and complications were minor with a lymph fistula in a single patient which was treated by surgery.


(2013) Knee dermolipectomy is a simple procedure with good results and few complications that improves the quality of life for obese patients. http://www.ncbi.nlm.nih.gov/pubmed/23254908

Transcutaneous Electrical Stimulation: A Case Report

Dercum’s disease is a rare condition of painful subcutaneous growth of adipose tissue. Etiology is unknown and pain is difficult to control. We report the case of a 57-year-old man with generalized diffuse Dercum’s disease, who improved after the treatment with transcutaneous frequency rhythmic electrical modulation system (FREMS).

Treatment consisted in 4 cycles of 30 minutes FREMS sessions over a 6-month period. Measures of efficacy included pain assessment (visual analogue scale, VAS), adipose tissue thickness by magnetic resonance imaging, total body composition and regional fat mass by dual-energy X-ray absorptiometry, physical disability (Barthel index), and health status (Short Form-36 questionnaire).

After FREMS treatment the patient’s clinical conditions significantly improved, with reduction of pain on the VAS scale from 64 to 17 points, improvement of daily life abilities (the Barthel index increased from 12 to 18) and amelioration of health status (higher scores than baseline in all Short Form-36 domains). Furthermore, we documented a 12 mm reduction in subcutaneous adipose tissue thickness at the abdominal wall and a 7040 g decrease in total body fat mass.


Full Text:

*Case reports are generally considered a type of anecdotal evidence. Given their intrinsic methodological limitations, including lack of statistical sampling, case reports are placed at the foot of the hierarchy of clinical evidence, together with case series. https://en.wikipedia.org/wiki/Case_report

Dercum’s Disease – A Mimic of Fibromyalgia

Authors: Gihyun Myung, M.D., and Meike A. Fang, M.D.

Case Report

A 40-year-old Caucasian male with a history of depression, alcohol abuse, and traumatic splenectomy to the Rheumatology Clinic for management of chronic body pain that started about 12 years ago. He reported having sharp, intermittent shoulder, finger, knee, and hip pain not alleviated by acetaminophen, thermal modalities, or physical therapy. He also noted numbness and tingling of his left arm and left leg. He was prescribed amitriptyline and bupropion for depression; these medications did not alleviate his pain. The patient was previously told he had psychosomatic pain by a neurologist.

His vital signs were normal, and he weighed 149 lbs with a BMI of 22.0. His physical examination was remarkable for multiple small nodules on the trunk and extremities and tenderness in the right supraspinatus and right trapezius muscles as well as the bilateral C7 region. Neurological examination was normal. Laboratory evaluation including Westergren erythrocyte sedimentation rate, C-reactive protein, thyroid function tests, antinuclear antibody, and rheumatoid factor were unremarkable. The initial impression was that the patient had fibromyalgia, peripheral neuropathy, or a chronic pain syndrome. Amitriptyline was switched to nortriptyline, and an electromyogram and nerve conduction study of the left arm and left leg were performed and were normal.

At his follow-up visit to the Rheumatology Clinic three months later, the patient reported that he developed multiple lumps in his trunk and extremities about 10 years ago, which were painful to touch. He reported prior biopsy of one of the nodules revealed “fatty tumor”. Repeat examination was notable for mobile, soft tender nodules on the trunk, bilateral arms extending from the antecubital fossa up to axillae and bilateral thighs. MRI of the right thigh showed tiny subcutaneous soft tissue nodular densities in the anterior medial proximal thigh.

His clinical presentation raised the possibility of Dercum’s disease, so he was referred to a physician who specialized adipose tissue disorders. She confirmed that he had Dercum’s disease type 3 and prescribed a diet high in omega-3 fatty acids and recommended analgesics such as gabapentin, pregabalin, or meloxicam.


Dercum’s disease (DD), also called adiposa dolorosa, is a rare condition characterized by painful subcutaneous lipomas. There are three types of DD: Type 1 involves joints, classically the knees; Type 2 is diffuse and generalized; Type 3 is nodular, which causes intense pain around multiple “lipomas”.

The pain is chronic (>3 months), symmetrical, often disabling. Even though prevalence is not known yet, DD is five to thirty times more common in women than in men. It most commonly occurs in peri- and post-menopausal women between the ages of 40 and 60 years and is characterized by lipomas involving the trunk and extremities. The presence of pain, which is required for diagnosis, is most commonly described as a burning or aching sensation. Usually, patients complain of pain that appears out of proportion to physical findings, but pain can range from hyperalgesia, discomfort on palpation, to paroxysmal attacks of pain. Even though obesity has been related with DD, it has been reported that people with normal weight can also have DD.

In addition to pain, there are reported associations with weakness, depression, confusion, lethargy, and dementia. The majority of the cases of DD occur sporadically, but some suggest DD might be an autosomal dominant disorder with variable expression. The differential diagnoses of multiple lipomas include fibromyalgia, benign symmetric lipomatosis, neurofibromatosis Type 1 (NF1), diffuse lipomas, familiar multiple lipomatosis, panniculitis, and congenital lipomatosis. It is important to note that DD is a clinical diagnosis and a diagnosis of exclusion.

The exact etiology of DD is unknown. Some early reported cases suggested treating thyroid disorders decreased pain raising the possibility that thyroid dysfunction is associated with DD. However, DD generally continues to progress even if thyroid replacement treatment is started, and recent studies have not revealed any endocrine laboratory abnormalities in patients with DD.

Helpful imaging modalities include ultrasound and MRI. Power Doppler reveals superficial subcutaneous lesions and absence of surrounding edema or increased vascularity. Compared to lipomas, DD nodules are smaller (mostly <2cm) and more hyperechoic. On MRI, lipomas generate high signal intensities on both T1- and T2-weighted images without enhancement after gadolinium injection. Ill-defined nodular/blush-lesions sometimes may be seen on unenhanced MRI. MRI provides accurate information regarding the relationship of the DD lesions to the surrounding tendons, bones, and joints and detects lesions that are not clinically obvious or symptomatic. Unlike lipomas, which can occur anywhere in the subcutaneous fat, DD lesions are located in the superficial subcutaneous fat.

DD does not have defining histological features; the biopsy of lipomas in DD is histologically identical to sporadic lipomas. Other studies have reported inflammatory changes and angiolipoma-like features. The cause of the pain in DD is unclear, but it may be due to pressure on nerves by the lipomas or increased vascularity, fibroblast proliferation, and fat cell necrosis around the nerve.

There are standard recommendations that could be tried. Unfortunately, non-steroidal, anti-inflammatory drugs have little for no effect. Intravenous lidocaine (400 mg over 15 mins every other day) or daily oral mexiletine have shown to provide pain relief. However, the provider should consider drug-drug interactions with concomitant medications and potential side effects when prescribing these medications. If these medications do not adequately provide pain relief, pregabalin, amitriptyline, or SSRIs could be tried.

Another class of medications that has been suggested is corticosteroids, typically oral prednisone. A single female patient’s pain was improved with Infliximab and methotrexate, and two patients with chronic hepatitis C were successfully treated with interferon alpha-2B. Non-pharmacologic approaches such as exercise, cognitive behavioral therapy, hypnosis, biofeedback, relaxation, transcutaneous electrical nerve stimulation, and distractions can be used as adjuncts to pharmacologic therapy. Liposuction can be also tried and is considered similar in efficacy compared to the surgical excision of the lipomas.


DD is a rare condition and a diagnosis of exclusion. Although it is more common in obese women of middle age, it can occur in males and those who have normal BMI. Due to overlapping symptoms, DD is commonly mistaken for fibromyalgia. It is important to distinguish DD from fibromyalgia since management differs. Fibromyalgia is a condition with widespread muscle pain and a painful response to pressure. Even though distinguishing features between fibromyalgia and DD can be very subtle, DD is suspected if patient has the multiple painful nodules in subcutaneous tissue. Because pharmacotherapy alone is often unsuccessful, it is important to have a multidisciplinary approach encompassing medication, surgery, and mental health.


*Case reports are generally considered a type of anecdotal evidence. Given their intrinsic methodological limitations, including lack of statistical sampling, case reports are placed at the foot of the hierarchy of clinical evidence, together with case series. https://en.wikipedia.org/wiki/Case_report

Ketamine Infusions (case study)

Ketamine infusion for refractory pain in Dercum’s Disease (Adiposis dolorosa): a novel treatment approach.

Dercum’s disease (Adiposis dolorosa) is a rare disorder characterized by multiple, tender subcutaneous nodules on the trunk and extremities. There is a higher incidence in obese, postmenopausal women. The exact mechanism is unknown, but it is hypothesized to be due to abnormal blood flow or circulatory dysfunction. Treatment is extremely challenging and is often times resistant to numerous modalities. We present the case of a 47 year-old female with a 2-year history of Dercum’s disease. The patient had seen numerous physicians and tried multiple treatment modalities, all with little success. After initial consultation, the decision was made to proceed with 2 four-hour trials of IV Ketamine infusions in the ICU. Patient reported approximately 60% relief of her pain immediately after her infusions and states that the relief lasted through her follow up four weeks later. Dercum’s disease remains a devastating diagnosis for many patients. It is extremely challenging to treat and has a significant adverse effect on patients’ quality of life. Ketamine infusions should be considered after conservative methods of treatment have failed and prior to proceeding to invasive treatment solutions such as liposuction.


*Case reports are generally considered a type of anecdotal evidence. Given their intrinsic methodological limitations, including lack of statistical sampling, case reports are placed at the foot of the hierarchy of clinical evidence, together with case series. https://en.wikipedia.org/wiki/Case_report

Liposuction (a literature review)

In dercum’s disease, Hansson et al. suggested liposuction may alleviate pain; however, it was difficult to determine whether this was due to the actual surgery or to other factors, according to EL-Khatib. De Silva and Earley used liposuction in two dercum’s patients and recommended the treatment as it had a low morbidity and was well-tolerated in elderly patients.

Treatment of this condition is usually targeted on pain relief rather than lipoma removal. El-Khatib reported a lack of scientific data for treatment or prevention of dercum’s disease.





%d bloggers like this: