Prednisone in the management of idiopathic hereditary lymphedema (Milroy's disease).

Idiopathic hereditary lymphedema may have its onset either at birth or at any time between childhood and early adulthood. Certain authors insist that the eponym "Milroy's disease" should be restricted to those cases that have a congenital origin, 1 whereas others see no need for undue emphasis on the age at which the edema appears, inasmuch as the clinical picture is otherwise essentially identical. 2 Milroy himself, in a 35-year follow-up report of the family he originally described, indicated that the age at which edema appears varies. 3 The prevailing opinion appears to be well summarized as follows 4 : "Primary lymphoedema is occasionally congenital, but more often it starts in childhood or adolescence and is then called lymphoedema praecox. In its most severe form the disease may produce deformities like those of tropical elephantiasis, and the term elephantiasis nostras (meaning 'ofour country') is then sometimes used." Families have been described