Dercum's Disease Research Articles

Multiple Ipsilateral Lower Extremity Masses in a 46-year-old Man

Multiple Ipsilateral Lower Extremity Masses in a 46-year-old Man

Dercum's Disease

Dercum's disease (adiposis dolorosa, lipomatosis dolorosa morbus Dercum), is a rare disorder resulting in painful fatty deposits around the upper legs, trunk, and upper arms. The portrait painted of Dercum's disease is very complicated, with many other disorders seen associated with the disease. There are no clear pathological mechanisms known, although it is suspected that there is either a metabolic or autoimmune component involved. Here, the authors review the literature to date, including some information from their own studies. In particular, the authors will look at the different strands of evidence pointing to the pathological mechanism of the disorder.

Maladie de Dercum : une complication grave au cours d'une maladie rare. À propos d'un cas

Dercum's disease or adiposis dolorasa is unusual and unknown. Four symptoms are typical. It is characterized by painful subcutaneous fatty tumors in postmenopausal woman. We present this disease through the study of an uncommon clinical case: a sceptic choc following the “steato-cutaneous-necrosis” of a fatty tumor. The disease remains rare. The etiology is to this day unknown and different hypothesis (endocrinous, metabolic, genetics) are put forward. Multiple complications can occur but severe septicemia is rare. The treatment can be medical (loss of weight, corticoids, intravenous lidocaine) or surgical (surgical excision or liposuction).

Dercum's Disease(Adiposis Dolorosa): Delayed Diagnosis in a Patient with Cervical Cancer

Dercum's Disease<i>(Adiposis Dolorosa)</i>: Delayed Diagnosis in a Patient with Cervical Cancer

Familial occurrence of adiposis dolorosa

Adiposis dolorosa is a rare condition characterized by multiple painful lipomas. We describe two typically affected siblings who were found to have at least 5 affected first-degree relatives in two generations. Manifestations were remarkably variable, ranging from complete disability to solitary asymptomatic tumors. Review of histopathologic findings did not reveal any features that might distinguish the tumors from common sporadic lipomas. Mutational analysis excluded the 8344 A to G mitochondrial mutation seen in other patients with multiple lipomas. Adiposis dolorosa may be an extreme manifestation of the more common condition of familial multiple lipomas. Further work is needed to define the genetic basis of these conditions. (J Am Acad Dermatol 2001;44:132-6.)

Liposuction in Dercum's disease: impact on haemostatic factors associated with cardiovascular disease and insulin sensitivity.

To study the impact of adipose tissue removal by liposuction on factors associated with increased risk of cardiovascular atherosclerotic disease within the coagulation and fibrinolytic system and glucose metabolism. Liposuction was performed in 53 patients with Dercum's disease. The levels of fibrinogen, von Willebrand factor antigen (VWF:Ag) and plasminogen activator inhibitor type 1 activity (PAI-1) were measured preoperatively, and 2 weeks, 4 weeks and 3 months postoperatively. In a subsample of 10 patients, insulin sensitivity was determined before and 2-4 weeks after surgery using the 2-h euglycaemic hyperinsulinaemic clamp technique. The study was performed as a single-centre study. Fibrinogen, PAI-1 and VWF:Ag levels, and glucose uptake before and after removal of adipose tissue. Weight reduction was sustained throughout the follow-up period with a mean decrease from 90.7 to 86.6 kg (P < 0.0001). There was a slight increase in levels of coagulation factors 2 and 4 weeks postoperatively, probably in reaction to the surgical trauma. After 3 months the values had returned to preoperative levels except for PAI-1, which still showed a slight increase (P < 0.05). In the subsample of 10 patients, glucose uptake was improved (P < 0.05) from a short-term perspective after surgery. Surgical removal of adipose tissue, without change in lifestyle, does not seem to improve the levels of coagulation and fibrinolytic factors associated with cardiovascular atherosclerotic disease, whereas glucose takeup may be facilitated and insulin sensitivity increases from a short-term perspective.

A pain in the groin

334 Vol 350 • August 2, 1997 Computed tomograph scan showing a large anterior abdominal wall “lipoma” (arrows) A 64-year-old woman with Dercum’s disease (adiposis dolorosa) attended the gynaecological clinic in February, 1996, with a 4-week history of lower left-sided abdominal and pelvic pain. An ultrasound scan showed uterine fibroids and she underwent hysterectomy and bilateral salpingo-oophorectomy. This did not relieve her symptoms and in July, 1996, she consulted her general practitioner with worsening pain. There was an obvious sausage-shaped lump in the left iliac fossa. She was reassured that this was another lipoma. 4 months later she was still in pain and had frequent bowel motions. A colonoscopy was normal and she was again reassured, as she was not keen to have the lipoma removed. She came back 3 months later with increasing discomfort, and at this time an ultrasound scan, requested to assess the size and position of the lump, failed to identify it. A computed tomograph scan was reported as showing a large abdominal wall lipoma (figure). She was offered surgical excision again, but declined. Despite further reassurance her symptoms got worse.

Jefferson Medical College: Legend and Lore

An extraordinarily engaging book,<i>Jefferson Medical College: Legend and Lore</i>is a labor of love to "record an institution's tradition and history" as its President, Paul C. Brucker, writes in the foreword. I was doubtful about the tome, the third in the trilogy of Jefferson's history, but it is beguiling to dip into its 15 chapters. They cover everything from the founding of the school, medical student life in the 19th century, alumni who founded other medical colleges, unusual Jefferson alumni, alumni who attended US presidents, eminent professors, and much more. I knew that the DaCostas were graduates of "Jeff," that Chevalier Jackson and Hobart Reimann had graced its corridors, but that Dercum of "Dercum's Disease" and John Gibbon of the heart-lung machine had worked there was news. There are chapters on early black alumni (the first identifiable black graduate, Algernon Jackson, graduated in 1901) and on early Jewish graduates

Adiposis Dolorosa (Dercumʼs Disease): 10-Year Follow-up

Adiposis dolorosa is a disease characterized by painful, subcutaneous fatty tumors. This disorder usually occurs in obese, postmenopausal women and is associated with weakness and mental disturbances such as depression, confusion, lethargy, and dementia. The cause is unknown, and there is no specific treatment. Pain may be relieved by steroids, intravenous lidocaine, or analgesics. Surgical treatment consists of excision or liposuction of the painful masses. We present two cases of adiposis dolorosa in men, with a follow-up of more than 10 years.

Fat-cell heat production, adipose tissue fatty acids, lipoprotein lipase activity and plasma lipoproteins in adiposis dolorosa.

1. Gluteal adipose tissue was examined in 13 patients with generalized adiposis dolorosa, a clinical condition characterized by painful adiposity with a chronic intractable course. The total metabolic activity of fat cells, isolated by collagenase and suspended in Krebs-Ringer bicarbonate buffer with glucose and insulin, was assessed by the measurement of heat production at 37 degrees C using microcalorimetry. 2. Fat cells were markedly enlarged; their metabolic activity expressed in terms of microW/g, but not in pW/cell, was significantly decreased when compared with both lean and weight-matched non-painful subjects. Both mean values were, however, significantly higher than in grossly obese subjects with similar mean cell size. Heat production as expressed per g of tissue, but not per cell, was inversely correlated with body mass index. One additional patient had unilateral disease, and fat cells from the painful side had a lower heat production than cells from the unaffected side. 3. The fatty acid composition of adipose tissue, as determined by g.c., revealed a significantly increased proportion of monounsaturated (18:1 and 16:1) at the expense of saturated (14:0 and 18:0) fatty acids compared with healthy control subjects. The activity of adipose tissue lipoprotein lipase was slightly, but not significantly, decreased. 4. It is concluded that a metabolic pathogenetic factor cannot be ruled out in adiposis dolorosa. As the results do not explain the nature of the diffuse pain, further studies need to be performed.

Corticosteroid-Induced Juxta-articular Adiposis Dolorosa

Long-term treatment with high doses of corticosteroids leads to the development of truncal obesity and focal fatty deposition. These deposits characteristically are located on the face, the nuchal and truncal areas, and episternally, as well as in the mediastinum and epicardium. We studied a patient with juxta-articular adiposis dolorosa who had L-tryptophan-associated eosinophilia-myalgia syndrome and was treated with high doses of prednisone. This is the first reported case of adiposis dolorosa occurring as a complication of corticosteroid treatment. Alterations of fat metabolism induced by corticosteroid excess may have played a role in the development of this unusual painful syndrome.

Liposuction in the treatment of juxta-articular adiposis dolorosa.

Two patients are presented who had symptomatic relief of juxta-articular adiposis dolorosa of the knees after liposuction. Liposuction is recommended for the treatment of this condition.

Adiposis Dolorosa (Dercumʼs Disease)

The clinical picture of adiposis dolorosa makes a lasting impression on the examining physician. The patient is typically an obese, asthenic woman who appears to have a low pain threshold. She has an unusual distribution of fatty tumors, and her complaint of pain in these tumors seems out of proportion to the physical findings. Alcoholism, emotional instability, and depression are common, and narcotic pain medicine is frequently requested. The patient is easily dismissed as a malingerer after a brief examination. However, liposuctioning of the painful fatty tumors appears to be both practical and effective. While adiposis dolorosa is an unusual disease, it is one that plastic surgeons can recognize and treat.

Juxta-articular adiposis dolorosa associated with rheumatoid arthritis. Report of 2 cases with good response to local corticosteroid injection

Juxta-articular adiposis dolorosa, i.e., painful fatty deposits around the joint may occur in postmenopausal obese women. The association of the syndrome with osteoarthritis has been described. The present report describes 2 women suffering from rheumatoid arthritis with painful fatty deposits around the knees. Both responded to local injection of corticosteroid. The possible pathogenesis of the source of pain is discussed.

Dercum's disease (adiposis dolorosa). Treatment of the severe pain with intravenous lidocaine

Dercum's disease (adiposis dolorosa) is associated with very painful subcutaneous fatty deposits normally localized to the lower extremities in which pain treatment is usually unsuccessful. Two patients with Dercum's disease have been treated repeatedly with intravenous lidocaine for several months each time resulting in immediate pain relief for 8 and 25 days respectively. Placebo infusions did not provide pain relief. Hereafter, the patients were treated with peroral mexiletine and again had complete pain relief. We conclude that intravenous lidocaine or peroral mexiletine may be an effective analgesic treatment in patients with Dercum's disease.

Long-standing pain relief of adiposis dolorosa (Dercum's disease) after intravenous infusion of lidocaine

A 60-year-old woman with adiposis dolorosa for 20 years was treated with repeated intravenous infusions of lidocaine. Partial relief of pain in the legs was obtained after 1.3 gm. A dose of 5.2 gm lidocaine given for 4 days was needed for complete pain relief. The effect lasted for 3 weeks, and then the pain gradually returned. The patient has now been given two additional treatments with complete pain relief for 2 months. The mechanism of the effect remains unknown.

The regulation of subcutaneous blood flow in patient with Dercum´s disease

Dercum´s disease or adiposis dolorosa is a poorly understood disorder with painful fatty deposits in the skin localized to the lower extremities. The etiology is unknown. In such a patient the mechanisms of local regulation of blood flow in subcutaneous tissue was investigated by the local 133Xenon washout technique. The patient was reinvestigated one week after treatment with intravenous lidocaine. The local vasoconstrictor response to increase in venous transmural pressure was not present in this patient, but reappeared after lidocaine treatment. Autoregulation of blood flow in subcutaneous tissue was present before as well as after lidocaine treatment. It seems likely that a pain elicited increase in sympathetic activity in the vasoconstrictor fibres abolished the normal vasoconstrictor response to increase in venous transmural pressure. The mechanism of pain relief after intravenous lidocaine infusion is uncertain, but central as well as peripheral mechanisms may be considered.

Dercum??s Disease (Adiposis Dolorosa)

Dercum's disease consists of multiple, painful lipomata and occurs in obese, postmenopausal women. A 52-year-old obese woman complained of multiple painful "lumps" in the extremities. The diagnosis of Dercum's disease (adiposis dolorosa) was established by excisional biopsy of four tumors. There is no effective treatment for this condition. Surgical excision, in this case, relieved the patient's symptoms locally, but after one year the painful lipomata had appeared at other sites. Case reports of this condition were not found in a review of the orthopedic literature.

Dercum's disease

Dercum's disease

Autosomal dominant inheritance in adiposis dolorosa (Dercum's disease).

A family in which 5 members were affected with adiposis dolorosa (Dercum's disease) is reported. The pedigree analysis gives evidence of autosomal dominant inheritance.