[Primary lymphedema]

Abstract

Secondary peripheral lymphedemas (all edemas based on pontine symptoms) are very much more frequent than primary edemas which are either congenital or due to a predisposition. Of the latter, only hereditary (essential) lymphedema and idiopathic lymphedema (lymphedema praecox) play a role in clinical practise with the regard to their incidence. The exceedingly rare hereditary lymphedemas, which differ from the primary noncongenital lymphedemas only with regard to the demonstrated heritability merely have a clinical significance. Thus for example only six such cases occurred amongst our patients in the last 20 years. Most primary lymphedemas occur at the time of puberty with a time span of 10 to 30 years. Primary lymphedemas can also occur within a genuine clinical picture (flat nevi, Recklinghausen disease etc.), so that underlying symptoms must be looked for when they occur.