Treatment of patients with hereditary lymphedema is primarily directed against the prevention of infection and the control of local

complications of limb swelling. Studies in mice, however, suggest that induced overexpression of VEGFR3 ligands stimulate the growth of functional lymphatic vessels.[13] An increase in lymphatics would benefit patients with primary and secondary lymphedema.

Exercise and elevation of the extremity promote movement of fluid away from the lower extremity. Elastic stockings and bandages are applied in a gradient fashion, with the highest compression distally and no constriction points. Gentle massage or pneumatic compression can be used. Proper skin hygiene and use of skin moisturizers prevents drying and fissuring of skin and subsequent bacterial skin infections. Use an antistreptococcal antibiotic to treat recurrent cellulitis. Prophylactic antibiotic therapy, such as benzathine penicillin in low doses, may be used to prevent intermittent cellulitis. Antibiotics directed at prophylaxis should have good activity against Streptococcus pyogenes. No medication treats or prevents Milroy disease. Complications of this disease, including cellulitis, bacteremia, and chylothorax, are treated as required. Benzopyrones, such as coumarin or diosmin, may stimulate proteolysis of tissue proteins.

Surgical Care

Several surgical methods have been attempted to benefit patients with Milroy disease, but none has achieved lasting success. Excision of the fibrotic subcutaneous tissues with coverage by split-thickness skin grafts has been described. Pedicular transfer of skin with healthy lymphatics to the affected limb and anastomosis to existing lymphatic channels has been tried without much success.

Consultations

Consultation with an infectious disease specialist is indicated for the treatment of recurrent cellulitis. In the future, consultation with a geneticist will provide insights into familial inheritance patterns.