Definition, Epidemiology and Pathophysiology of Lymphoedema

Lymphoedema is a physically and psychologically stressful, chronic progressive disease caused by long lasting damage or malfunction of the lymphatic drainage system. It is classified as primary when caused by a genetic predisposition (inherited or congenital) directly affecting any part of the lymphatic vascular system, or, much more often, as secondary (acquired) when caused by factors outside the lymphatic system, such as tumours or their treatment. As part of the development of an S3 guideline for the diagnosis and treatment of lymphoedema in German-speaking countries, we present here the definition of lymphoedema, its epidemiology, staging, pathophysiology, aggravating comorbidities, and differentiation from other forms of oedema or volume-increasing diseases. We refer to molecular links with obesity, present a diagram of possible pathomechanistic interactions, and finally discuss approaches for potential drug therapies. More intensive molecular genetic diagnostics of primary lymphedema seem to be gaining ground in Germany currently. We recommend further strengthening these diagnostics, as this is the only way to expand therapeutic options in the future and use existing therapies more efficiently.