The present, revised guidelines on lipedema were developed under the auspices of and funded by the German Society of Phlebology (DGP). The recommendations are based on a systematic literature search and the consensus of eight medical societies and working groups. The guidelines contain recommendations with respect to diagnosis and management of lipedema. The diagnosis is established on the basis of medical history and clinical findings. Characteristically, there is a localized, symmetrical increase in subcutaneous adipose tissue in arms and legs that is in marked disproportion to the trunk. Other findings include edema, easy bruising, and increased tenderness. Further diagnostic tests are usually reserved for special cases that require additional workup. Lipedema is a chronic, progressive disorder marked by the individual variability and unpredictability of its clinical course. Treatment consists of four therapeutic mainstays that should be combined as necessary and address current clinical symptoms: complex physical therapy (manual lymphatic drainage, compression therapy, exercise therapy, and skin care), liposuction and plastic surgery, diet, and physical activity, as well as psychotherapy if necessary. Surgical procedures are indicated if - despite thorough conservative treatment - symptoms persist, or if there is progression of clinical findings and/or symptoms. If present, morbid obesity should be therapeutically addressed prior to liposuction.

Surgical approaches are usually not part of the strand rad approach in lymphologic therapy. Classic therapy is conservative and controls symptoms rather than seeking cures. Plastic surgical tissue reduction results in impaired lymph flow in many cases. Improving the lymphologic disease while reducing the need for complex compression therapy are major therapeutic goals. Lymphologic liposculpture offers a successful way to treat lipohyperplasia dolorosa and offers a new concept in the treatment of secondary lymphedema.

Lipohyperplasia dolorosa and lymphedema are completely different disease entities, although both are classified under lymphology. Whereas in lipohyperplasia dolorosa a congenital lipid distribution disorder leads to a high volume insufficiency and corresponding clinical symptoms, lymphedema is characterized by a congenital transport incompetence of the vessels or acquired disorders of transport capacity. According to current knowledge the solid increase in volume with lymphedema is due to a malfunctioning bio-mechanism by which the release of additional proteoglycans in the homeostasis system of the fluid in the interstitial space plays an important role. Therapeutic approaches aim to remove this tissue and the sponge-like proteoglycan substance. Manual lymph drainage and compression can evacuate the sponge but not remove it. Lymphological liposculpture has been successfully used to treat lipohyperplasia dolorosa by removing subcutaneous fatty tissue, present as hyperplasia and not hypertrophy. After lymphological liposculpture, tenderness is no longer present and complex decongestive therapy is no longer necessary because the congenital fatty masses do not recur after surgical removal. The improvement is thus permanent. Lipohyperplasia dolorosa is therefore curable by lymphological liposculpture. The procedure can even achieve a drastic improvement in quality of life for patients with secondary lymphedema by adjusting the symmetry of the extremities and reducing or even avoiding complex decongestive therapy.

Summary: Proteoglycans (PG) are essential for regulating water flow in the interstitium. From stage 1 of lymphostasis, there is an accumulation of interstitial PG, which regulate the increasing fluids. As the disease progresses, more PG are formed than degraded, resulting in proliferation, and increases in circumference and volume of solid tissue. The removal of this subcutaneous tissue, which is very rigid due to cross-linked PG, is a particular challenge in lymphedema surgery. Hyaluronidase has a lytic effect on these PG structures and, after subcutaneous infiltration, reduces the viscosity of the extracellular matrix, promoting diffusion and penetration of solutions into the surrounding tissue. By using hyaluronidase in our vascular-sparing surgical protocol (lymphological liposculpture), we have not observed any lymphedema recurrences even after 15 years.