Lipedema is a symmetrical disorder of the subcutaneous adipose tissue that affects almost exclusively women in postpubescent age. The trinity of disfiguring hyperplastic adipose tissue of the proximal extremities, increasing pain over time and bruising is characteristic. Lymphedema can occur in both sexes and is differentiated into primary and secondary subtypes. Symmetry is not a prerequisite for diagnosis. Characteristic for lymphedema is the disproportion between lymphatic fluid and the capacity of lymphatic vessels. The most distal body parts are always more severely affected than proximal parts. In the initial phases lymphedema is painless but in advanced stages tension pain can occur. The combination of both disorders has been described; however, lipedema is not responsible for subsequent lymphedema in contrast to central obesity, which significantly increases the risk of lymphedema. The differential diagnosis is of utmost importance for a meaningful management.

Lipedema is a symmetrical disorder of the subcutaneous adipose tissue that affects almost exclusively women in postpubescent age. The trinity of disfiguring hyperplastic adipose tissue of the proximal extremities, increasing pain over time and bruising is characteristic. Lymphedema can occur in both sexes and is differentiated into primary and secondary subtypes. Symmetry is not a prerequisite for diagnosis. Characteristic for lymphedema is the disproportion between lymphatic fluid and the capacity of lymphatic vessels. The most distal body parts are always more severely affected than proximal parts. In the initial phases lymphedema is painless but in advanced stages tension pain can occur. The combination of both disorders has been described; however, lipedema is not responsible for subsequent lymphedema in contrast to central obesity, which significantly increases the risk of lymphedema. The differential diagnosis is of utmost importance for a meaningful management.

Introduction Lipedema is a chronic, progressive condition that can result in considerable disability. In 2011, the Dutch Society of Dermatology and Venereology organized a task force to create guidelines on lipedema, using the International Classification of Functioning, Disability and Health of the World Health Organization. Guideline development Clinical questions on significant issues in lipedema care were proposed, involving (1) making the diagnosis of lipedema; (2) clinimetric measurements for early detection and adequate follow-up; and (3) treatment. A systematic review of literature published up to June 2013 was conducted. Based on available evidence and experience of the task force, answers were formed and recommendations were stated. The guidelines define criteria to make a medical diagnosis of lipedema, a minimum data set of (repeated) clinical measurements that should be used to ensure early detection and an individually outlined follow-up plan, pillars on which conservative treatment should be based and recommendations on surgical treatment options. Conclusions Little consistent information concerning either diagnostics or therapy can be found in the literature. It is likely that lipedema is frequently misdiagnosed or wrongly diagnosed as only an aesthetic problem and therefore under- or mis-treated. Treatment is divided into conservative and chirurgic treatment. The only available technique to correct the abnormal adipose tissue is surgery. Recommendations To ensure early detection and an individually outlined follow-up, the committee advises the use of a minimum data set of (repeated) measurements of waist circumference, circumference of involved limbs, body mass index and scoring of the level of daily practice and psychosocial distress. Promotion of a healthy lifestyle with individually adjusted weight control measures, graded activity training programs, edema reduction, and other supportive measures are pillars of conservative therapy. Tumescent liposuction is the treatment of choice for patients with a suitable health profile and/or inadequate response to conservative and supportive measures.