Deoxycholic Acid for Dercum Disease: Repurposing a Cosmetic Agent to Treat a Rare Disease.

Dercum disease is a rare condition characterized by multiple painful fatty tumors distributed throughout the body. There currently are no US Food and Drug Administration-approved treatments for Dercum disease, and the treatments tried have shown little to no efficacy, leaving many patients with a profoundly negative impact on quality of life. We present a case series of 3 patients who were diagnosed with Dercum disease and were treated with deoxycholic acid (DCA), a therapy approved for adipolysis of submental fat. The patients experienced a reduction in tumor size with radiographic evidence as well as a notable reduction in symptoms.

Lipedema and Dercum's Disease: A New Application of Bioimpedance.

Background: Lipedema and Dercum's disease (DD) are incompletely characterized adipose tissue diseases, and objective measures of disease profiles are needed to aid in differential diagnosis. We hypothesized that fluid properties, quantified as tissue water bioimpedance in the upper and lower extremities, differ regionally between these conditions. Methods and Results: Women (cumulative n = 156) with lipedema (n = 110), DD (n = 25), or without an adipose disease matched for age and body mass index to early stage lipedema patients (i.e., controls n = 21) were enrolled. Bioimpedance spectroscopy (BIS) was applied to measure impedance values in the arms and legs, indicative of extracellular water levels. Impedance values were recorded for each limb, as well as the leg-to-arm impedance ratio. Regression models were applied to evaluate hypothesized relationships between impedance and clinical indicators of disease (significance criteria: two-sided p < 0.05). Higher extracellular water was indicated (i) in the legs of patients with higher compared with lower stages of lipedema (p = 0.03), (ii) in the leg-to-arm impedance ratio in patients with lipedema compared with patients with DD (p ≤ 0.001), and (iii) in the leg-to-arm impedance ratio in patients with stage 1 lipedema compared with controls (p ≤ 0.01). Conclusion: BIS is a noninvasive portable modality to assess tissue water, and this device is available in both specialized and nonspecialized centers. These findings support that regional bioimpedance measures may help to distinguish lipedema from DD, as well as to identify early stages of lipedema.

Trauma-induced adiposis dolorosa (Dercum's disease).

We present a case of a 39-year-old man who presented with chronic bilateral upper extremity pain associated with innumerable angiomyolipomas that developed 5 years after a motor vehicle accident involving his upper extremities. Our case notes the rare nature of painful adipose tissue deposits and the diagnostic challenges.

Lipomatoses.

Lipomatoses are benign proliferations of adipose tissue, often with typical distribution patterns, which usually occur without clear causes. In contrast to circumscribed lipomas, they develop diffusely and symmetrically and are not surrounded by a fibrous pseudocapsule. The most common form is benign symmetric lipomatosis (BSL; Launois-Bensaude syndrome), of which four types are distinguished based on the distribution pattern of hyperplastic adipose tissue. The etiology and pathogenesis of the disease are still largely unknown, although some forms appear to have a hereditary basis or to be associated with increased alcohol consumption. In some cases, mitochondrial dysfunctions have been detected. Lipomatoses may be solitary, but may also be associated with other symptoms or illnesses (comorbidity). Effective drug therapies are not known, but surgical procedures can alleviate the symptoms. Lipomatosis dolorosa, which can be distinguished from BSL, occurs predominantly in middle-aged women and is characterized by proliferation of adipose tissue and severe pain in the affected areas. Various differential diagnoses and concomitant illnesses need to be considered, in particular depression. There are no evidence-based recommendations for therapy. Repetitive lidocaine infusions can achieve temporary improvement. Recurrence after surgical intervention is not uncommon, but long-term success is also possible.

[Skin manifestations, treatment and rehabilitation in overweight and obesity]. / A túlsúly és az elhízás bortünetei, kezelés, rehabilitáció

Overweight and obesity is a public health problem in Hungary and in the Western world. It is important to underline that obesity is an illness and an important risk factor for several skin and other diseases. An overview of skin diseases caused or aggravated by obesity (acanthosis nigricans, acrochordons, keratosis pilaris, hyperandrogenism, stria, adiposis dolorosa, lymphoedema, chronic venous insufficiency, plantar hyperkeratosis, lipoedema, skin infections, acne inversa, psoriasis, tophi) helps us to look and see as well. Look for the possibility of skin infections as it helps the early diagnosis and to avoid complications. Draw patients' attention to the preventive importance of skin care. In case of an obese patient the usual dosage of most local and systemic drugs should be modified. It must be kept in mind that obesity directly or indirectly starts unfavorable processes in almost all organ systems. Therefore, only a multidisciplinary care may secure treatment and rehabilitation of obese patients. Dermatological and lymphological care is often part of the rehabilitation.

[Dercum's disease: a severe complication in a rare disease. A case report]. / Maladie de Dercum: une complication grave au cours d'une maladie rare. A 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.

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.

Adiposis dolorosa (Dercum's disease): liposuction as an effective form of treatment.

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.

A case of adiposis dolorosa: lipid metabolism and hormone secretion.

The present report describes a 53-year-old non-obese man with adiposis dolorosa whose pain was dramatically relieved by the intravenous injection of lidocaine. The patient showed a paradoxical response of growth hormone to thyrotropin-releasing hormone. In addition, in-vitro studies on adipose tissue metabolism revealed the reduced glucose conversion to neutral glycerides in painful adipose tissue. These abnormalities may be related in some ways to the pathogenesis of this disorder.

Adiposidade dolorosa justa-articular dos joelhos / Adiposis dolorosa juxta-articular of knees

Cem mulheres com queixas de dor no joelho foram prospectivamente estudadas. Entre elas foram encontrados 12 casos de adiposidade dolorosa justa-articular dos joelhos. Esta patologia é identificada pela presença de adiposidade dolorosa na face medial de ambos os joelhos e deve ser entendida como uma entidade perfeitamente individualizada. Nossas pacientes apresentavam as seguintes características básicas: obesidade, pé plano, joelho valgo e história familiar de adiposidade dolorosa. De acordo com os nossos dados e com os da literatura, a adiposidade dolorosa justa-articular pode, talvez, ser incluída no espectro da doença de Dercum