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Lipedema is an underdiagnosed syndrome of unclear etiology, characterized by symmetric painful enlargement of the buttocks and lower extremities, which spares the feet. This enlargement is caused by the deposition of adipose tissue. It was first described by Allen and Hines in 1940, who observed that it had a female predilection; patients commonly had an associated family history. We describe a patient with classic lipedema and multiple lipomas of her arms and trunk.

BACKGROUND AND PURPOSE: Unclear extremity complaints are common symptoms of inpatients. In a subset of these patients, a clinical suspicion of deep vein thrombosis (DVT) results; this needs to be quickly and definitively clarified by a vascular physician. The question arose of how often a clinical suspicion of DVT was confirmed in an inpatient population and which alternative diagnoses were able to be made by angiologists. PATIENTS AND METHODS: In a retrospective analysis, all inpatients in the Angiologic Vascular Diagnostics Center of the University Hospital Halle, Germany, examined in 2007 for a suspicion of DVT were evaluated with respect to the definitively made diagnosis. RESULTS: In 213 (28.6%) of 745 suspected cases of DVT, a DVT was confirmed. In 532 patients (71.4%), DVT was excluded. In 314 of these patients, 436 alternative diagnoses were recorded in the diagnostic reports of angiologic examinations. In 38.6% (n = 168), other venous causes could be confirmed as the most common alternative diagnosis. There were chronic venous diseases in 28% (n = 122), superficial thrombophlebitis (n = 27), and tumor-related pelvic vein compression (n = 19). 17.4% (n = 76) exhibited lymphedema. In 13.3% (n = 58), a generalized edema was diagnosed. Arthrogenic causes followed with 12.8% (n = 56). Lipedema (5.3%) and hematoma (5%) could be verified as other important differential diagnoses. Rare causes were symptomatic or ruptured Baker's cysts (2.5%), erysipelas (2.5%), abscess, aneurysm, muscle tears, and tumors. CONCLUSION: The variety of alternative diagnoses in patients with clinical suspicion of DVT is high. The knowledge and systematic examination of potential, even rare differential diagnoses after exclusion of DVT are part of the repertoire of the vascular physician. Unnecessary and expensive, as well as onerous, diagnostic procedures on the patient can be avoided. Anticoagulation that was begun as a result of the suspicion of DVT can quickly be stopped.

Adiposis dolorosa (AD) is a rare disorder of painful nodular subcutaneous fat accompanied by fatigue, difficulty with weight loss, inflammation, increased fluid in adipose tissue (lipedema and lymphedema), and hyperalgesia. Sequential compression relieves lymphedema pain; we therefore hypothesized that whole body cyclic pneumatic hypobaric compression may relieve pain in AD. To avoid exacerbating hyperalgesia, we utilized a touch-free method, which is delivered via a high-performance altitude simulator, the Cyclic Variations in Altitude Conditioning™ (CVAC™) process. As a pilot study, 10 participants with AD completed pain and quality of life questionnaires before and after 20-40 minutes of CVAC process daily for 5 days. Participants lost weight (195.5 ± 17.6-193.8 ± 17.3 lb; P = 0.03), and bioimpedance significantly decreased (510 ± 36-490 ± 38 ohm; P = 0.01). There was a significant decrease in scores on the Pain Catastrophizing Scale (P = 0.039), in average (P = 0.002), highest (P = 0.029), lowest (P = 0.04), and current pain severity (P = 0.02) on the Visual Analogue Scale, but there was no change in pain quality by the McGill Pain Questionnaire. There were no significant changes in total and physical SF-36 scores, but the mental score improved significantly (P = 0.049). There were no changes in the Pain Disability Index or Pittsburgh Sleep Quality Index. These data present a potential, new, noninvasive means of treating pain in AD by whole body pneumatic compression as part of the CVAC process. Although randomized, controlled trials are needed to confirm these data, the CVAC process could potentially help in treating AD pain and other chronic pain disorders.

BACKGROUND: Lipoedema is an accumulation of fat abnormally distributed in the lower limbs, and lymphoedema is oedema caused by a deficiency of the lymphatic system. High-resolution ultrasound operating at 20 MHz makes it possible to characterize dermal oedema. OBJECTIVES: The purpose of our study was to demonstrate that high-resolution ultrasound imaging of the skin can differentiate lipoedema from lymphoedema. METHODS: Sixteen patients with lymphoedema (22 legs), eight patients with lipoedema (16 legs) and eight controls (16 legs) were included. Patients with lipolymphoedema were excluded. Ultrasound examinations were carried out with a real-time high-resolution ultrasound device on three different sites for each lower limb. The images were then anonymized and examined by an independent dermatologist who was blind to the clinical diagnosis. A new series of images was examined by three dermatologists to check interobserver agreement. RESULTS: A significant difference in dermal thickness was observed between patients with lymphoedema and those with lipoedema and between patients with lymphoedema and controls. No significant difference in dermal thickness was shown between patients with lipoedema and controls at the thigh or ankle. Dermal hypo-echogenicity was found in at least one of the three sites in 100% of patients with lymphoedema, 12.5% of cases with lipoedema and 6.25% of the controls. Hypoechogenicity affected the entire dermis in all cases of lymphoedema except one. In cases of lipoedema and controls, hypoechogenicity was localized at the ankle and prevailed in the upper dermis. The expert correctly diagnosed all lower limbs with lymphoedema. No cases of lipoedema were diagnosed as lymphoedema. Exact interobserver agreement was excellent (0.98). CONCLUSIONS: High-resolution cutaneous ultrasonography makes it possible to differentiate lymphoedema from lipoedema. Obtaining a reliable diagnosis through high-resolution cutaneous ultrasonography might be valuable for improving the treatment of lipoedema and lymphoedema.

BACKGROUND: A 23-year-old male was referred to our clinic with diagnosis of idiopathic isolated growth hormone deficiency. A detailed family history revealed short stature and swelling of legs which only affected females in four generations of his family. METHODS: Combined pituitary function tests revealed growth hormone deficiency, secondary hypothyroidism and hypoprolactinemia in the proband. His mother had hypoprolactinemia and growth hormone deficiency. A diagnosis of inherited combined pituitary deficiency due to a PIT-1 mutation was suspected in view of the short stature with associated multiple pituitary hormone deficiencies. RESULTS: A mutation was identified in PIT-1 (POU1F1), 196C>T, which produces the amino acid change P24L in exon 1. The mutation was also found in the mother of the proband but not in his phenotypically normal half-sister. CONCLUSION: The case shows a novel association of two rare conditions Pit-1 mutation and lipoedema in a family that has not been described before. It also allows formulation of hypothesis on the interaction of growth hormone and sex steroids resulting in abnormal fat distribution in predisposed subjects at the time of puberty.

Lipedema is a condition characterized by swelling and enlargement of the lower limbs due to abnormal deposition of subcutaneous fat. Lipedema is an under-recognized condition, often misdiagnosed as lymphedema or dismissed as simple obesity. We present a series of pedigrees and propose that lipedema is a genetic condition with either X-linked dominant inheritance or more likely, autosomal dominant inheritance with sex limitation. Lipedema appears to be a condition almost exclusively affecting females, presumably estrogen-requiring as it usually manifests at puberty. Lipedema is an entity distinct from obesity, but may be wrongly diagnosed as primary obesity, due to clinical overlap. The phenotype suggests a condition distinct from obesity and associated with pain, tenderness, and easy bruising in affected areas.

Lipoedema is a distinct clinical condition characterized by bilateral, symmetrical enlargement of the buttocks and lower limbs owing to excess deposition of subcutaneous fat. It is found almost exclusively in women. The common features associated with this condition are 'column- shaped' legs with sparing of the feet, bruising, sensitivity to pressure, and orthostatic oedema. The progression to lipo-lymphoedema or morbid obesity is possible. Conservative measures used in the management of lymphoedema can prevent progression/limit the orthostatic oedema. Surgical procedures may also play a part in the management of lipoedema.

Lipohyperplasia dolorosa and lymphedema are completely different disease entities, which are both, however, classified under lymphology. While in lipohyperplasia dolorosa a congenital lipid distribution disorder leads to a high volume insufficiency and the corresponding clinical symptoms, lymphedema is characterized by a congenital transport incompetence of the vessels or acquired disorders of transport capacity. Both lymphedemas of different genesis are familial volume alterations of the affected regions and the increase in volume is irreversible if not exclusively still in stage I or II. According to current knowledge the solid increase in volume by lymphedema is due to a malfunctioning biomechanism by which the release of additional proteoglycans in the homeostasis system of the fluid in the interstital space plays an important role. Removal of this tissue and the sponge-like substance of proteoglycans is the aim of therapeutic approaches. Manual lymph drainage and compression can evacuate the sponge but not remove it. Lymphological liposculpture is a successful dermatosurgical measure even for secondary lymphedema. Reduction of the necessity of complex hemostasis therapy to 20% of the initial value and an adjustment of the affected extremity on the healthy side, represent a clear improvement in quality of life of patients. The same dermatosurgical method, lymphological liposculpture, has been known for many years to fulfil the successfully proven purpose for the treatment of lipohyperplasia dolorosa by the removal of subcutaneous fatty tissue, present as hyperplasia and not hypertrophy. Tenderness and the necessity for complex hemostasis therapy are no longer present or no longer necessary after lymphological liposculpture for lipohyperplasia dolorosa. This condition is permanent because the congenital fatty masses do not reoccur following surgical removal. Lipohyperplasia dolorosa is therefore curable by lymphological liposculpture. For secondary lymphedema a drastic improvement in quality of life of the patient can be achieved by this method which is demonstrated by the adjustment of symmetry of the extremities and reduction or even avoidance of complex hemostasis therapy.

A prospective single-center study with mail questionnaire – in most cases combined with clinical controls – was carried out in 112 patients with lipedema after a period from 8 months to nearly 7 years following liposuction in tumescent local anesthesia.