Lymphatic scintigraphy was not performed and the diagnosis of primary lymphedema was made from clinical presentation. Primary causes of chronic lymphedema are classified as congenital, familial, or congenitally determined but of late onset. Milroy's disease is a rare form of familial lymphedema, manifesting soon after birth. Meige's disease is also familial but manifests in childhood or at puberty. Non-familial sporadic cases are more common and are seen frequently in women at or around menopause.
Diuretics are generally unhelpful except in reducing concomitant venous dependent edema.
This Image of Osler shows chronic unilateral primary lymphedema diagnosed in a woman who presented with worsening mobility 33 years after the onset of leg swelling. Unrelated to her presenting complaints was her long history of pulmonary sarcoid and chronic shortness of breath. There is also right paratracheal and anterior-posterior window adenopathy. This Image of Osler shows endstage pulmonary fibrosis in honeycomb lungs secondary to sarcoidosis.