Novel Use of Deoxycholic Acid for Adiposis Dolorosa (Dercum Disease).
“We describe a pair of female monozygotic twins with Dercum’s disease (DD) who presented simultaneously with unprovoked pulmonary emboli. Several genetic determinants have been associated with venous thromboembolism (VTE) but the overall influence of genetic factors is unknown. As yet there is no published evidence to support an increase in the risk of VTE in female monozygotic twins. DD is a rare condition characterized by multiple, painful lipomas. The underlying pathology of it is poorly understood. To date, there has been no recorded association with an increased risk of VTE but there have been reports of stroke-like events. It is unclear if these are caused by the condition itself or are co-incidental. We acknowledge the possibility of a coincidence but the two cases raise the question of an association between VTE and DD. This report should encourage further studies into the risk of VTE in female monozygotic twins and DD.”
“To our knowledge, this is the first report of a case of Dercum’s disease affecting a prepubescent child with lipomas in the dorsal region, face and neck, abdominal wall, arms and legs, which are common sites for lipomas seen in patients with Dercum’s disease in adulthood.” Full Text: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6248242/
Izar, Maria Cristina de Oliveira et al. “Rare Presentation of Dercum’s Disease in a Child with Abnormalities in Lipoprotein Metabolism” Arquivos brasileiros de cardiologia vol. 111,5 (2018): 755-757
Subcutaneous adipose tissue therapy reduces fat by dual X-ray absorptiometry scan and improves tissue structure by ultrasound in women with lipoedema and Dercum disease.
Lipoedema is painful nodular subcutaneous adipose tissue (SAT) on legs and arms of women sparing the trunk. People with Dercum disease (DD) have painful SAT masses. Lipoedema and DD fat resists loss by diet and exercise. Treatments other than surgery are needed. Six women with lipoedema and one with DD underwent twelve 90‐min sessions over 4 weeks. Body composition by dual X‐ray absorptiometry scan, leg volume, weight, pain, bioimpedance, tissue size by caliper and ultrasound were analysed before and after SAT therapy by paired t‐tests. There was a significant decrease from baseline to end of treatment in weight, 87.6 ± 21 to 86.1 ± 20.5 kg (P = 0.03), leg fat mass 17.8 ± 7.7 to 17.4 ± 7.6 kg (P = 0.008), total leg volume 12.9 ± 4 to 12 ± 3.5 L (P = 0.007), six of 20 calliper sites and tissue oedema. Pain scores did not change significantly. By ultrasound, six women had 22 hyperechoic masses in leg fat that resolved after treatment; five women developed seven new masses. Fascia improved by ultrasound after treatment. SAT therapy reduced amount and structure of fat in women with lipoedema and Dercum disease; studies are needed to compare SAT therapy to other therapies.
Adiposis dolorosa is a rare disorder of multiple painful subcutaneous growths of adipose tissue. It is also known as Dercum disease, Ander syndrome, morbus Dercum, adipose tissue rheumatism, adiposalgia, or lipomatosis dolorosa. This disease was first discovered in the late 1800s by American neurologist Francis Xavier Dercum. It is classified into 4 types which include generalized diffuse, generalized nodular, localized nodular, and juxta-articular forms. The generalized diffuse type presents with widespread painful adipose tissue with no apparent lipomas. The generalized nodular type presents with widespread, painful adipose tissue that is more painful in the vicinity of lipomas. In the localized nodular type, the pain is restricted to areas within and around lipomas. Lastly, the juxta-articular type presents as painful solitary adipose tissue near large joints. The diagnosis of adiposis dolorosa is made clinically and is a diagnosis of exclusion. The proposed criteria for the diagnosis includes chronic pain of the subcutaneous tissue for over 3 months in overweight or obese patient, though the criteria still need to be validated. The pain is often disabling and resistant to treatment. The disease is associated with weakness and psychiatric symptoms such as depression. Other associated symptoms include fatty deposits, easy bruising, sleep disturbances, impaired memory, difficulty concentrating, anxiety, rapid heartbeat, shortness of breath, diabetes, bloating, constipation, fatigue, and joint pain.
Read article at publishers site: https://www.ncbi.nlm.nih.gov/books/NBK507867/