Milroy disease presents at birth; the edema is confined to legs
and feet. Over time, with the development of fibrosis, the edema becomes
nonpitting. The overlying skin becomes hyperkeratotic with fissures, and
secondary infection occurs. Severe lymphedema is called elephantiasis (filariasis). Often associated
with congenital chylous ascites, recurrent scrotal swelling, intestinal
tract protein loss, persistent bilateral pleural effusion, and
hypoproteinemia. Poor wound healing after trauma. In adulthood, development
of lymphangiosarcomas and squamous epidermoid carcinomas, with 50%
mortality within 24 months of diagnosis. Normal life expectancy in patients
without tumors. Management is otherwise symptomatic; combined physical
therapy with tight bandages and stockings, massage, and use of pneumatic
devices for intermittent compression considerably reduces the edema and
renders surgery unnecessary in most patients. Diuretics have a beneficial
effect during early management and benzopyrones for long-term treatment.
Surgical excision of subcutaneous tissue followed by skin graft is performed
in selected patients.