In lymphedema (thesaurus synonyms: Congenital hereditary elephantiasis; Elephantiasis after mastectomy; Elephantiasis; Elephantiasis teleangiectodes; Glandular elephantiasis; Hereditary lymphedema; Hereditary trophedema; Testicular elephantiasis; Scrotal elephantiasis; Lymphangiectasia; Lymphangiectasia of the scrotum; Lymphangiectatic elephantiasis; Lymphatic obstruction; Lymphatic edema; Lymphectasia; Lymphatic vessel dilation; Lymphatic vessel dilation; Lymphatic vessel compression; Lymphatic vessel obstruction; Lymphatic vessel obstruction after mastectomy; Lymphatic vessel occlusion; Lymphatic vessel occlusion after mastectomy; Lymphedema; Lymphedema of the arm; Lymphedema of the leg; Lymphedema after ablatio mammae; Lymphedema after mastectomy; Lymphedema syndrome after mastectomy; Lymphoedema praecox; Meige’s disease; Nonne-Milroy disease; Nonne-Milroy-Meige syndrome; Upper arm lymphedema after mastectomy; Obstruction of the thoracic duct; Edema associated with lymphatic vessel obstruction; Orchelephantiasis; Orchelephantiasis; Penile elephantiasis; Penile elephantiasis; Postinfectious lymphangiectasia; Postmastectomy edema; Postmastectomy syndrome; Secondary lymphedema; Scrotum elephantiasis; Trophedema; Obstruction of the thoracic duct; ICD-10-GM I89. 0: Lymphedema, not elsewhere classified; ICD-10-GM I97.2: Lymphedema after mastectomy; ICD-10-GM Q82.0: Hereditary lymphedema) is a proliferation of tissue fluid caused by damage to the lymphatic system.
Primary lymphedema can be distinguished from secondary lymphedema:
- Primary lymphedema (hereditary lymphedema; congenital form; genetic predisposition).
- Secondary lymphedema (acquired form, i.e., as a result of, for example, diseases, injuries and therapies).
Secondary lymphedema is about twice as common as primary lymphedema.
Sex ratio: women to men is 9: 1.
Frequency peak: the maximum occurrence of lymphedema is between the ages of 15 and 20 (lymphoedema praecox/early lymphedema). In approximately 17% of cases, lymphedema occurs after the age of 35 (lymphoedema tardum/late lymphedema).
The prevalence (disease frequency) of lymphedema is about 1.8-3 %.The prevalence of primary lymphedema in Germany is estimated at 0.5 ‰. Of these, about 97% are sporadic and only about 3% are congenital lymphedema.
Course and prognosis: Lymphedema, if untreated, is a progressive (progressive), chronic disease as a result of primary or secondary damage to the lymphatic drainage system leading to the proliferation and alteration of interstitial tissue fluid (fluid located between tissues). As the disease progresses, tissue changes occur, with an increase in connective and adipose tissue and changes in the extracellular matrix (extracellular matrix, intercellular substance, ECM, ECM: glycosaminoglycans, hyaluronic acid, collagen).
