Hereditary lymphoedema, an often unrecognized form of chronic oedema; A clinical study in two families with Meige's disease

Two families with an autosomal-dominantly hereditary form of lymphoedema of the legs (Meige's disease) are described. The oedema only manifests itself after puberty, with highly variable age of onset, and is progressive. Once cardiac, renal and other causes of oedema have been excluded, lymphoscintigraphy is the best diagnostic technique; it renders lymphangiography superfluous for the demonstration of complete or partial absence of lymphatics. Emphasis is laid on the necessity of early recognition of this cause of oedema, adequate diagnostics and conservative therapy.