A 40-year-old man presented with a 5-year history of progressively increasing bilateral leg swelling that was neither painful nor pruritic. There was no history of dyspnea, jaundice, or groin surgery. The patient reported multiple previous episodes of superimposed cellulitis, which were treated with antibiotics. There was no family history of similar illness. The clinical examination for venous insufficiency was noncontributory. Drs Sajal Kumar and Shanmugam Uthamalingam of New Rochelle, NY, noted that the nonpitting edema of both legs with extensive excoriation, fibrosis, and hyperpigmentation was characteristic of lymphedema. The diffuse erythema and induration suggested superimposed cellulitis. Primary lymphedemas are often classified by the age of the patient at edema onset. The 3 forms of the disease are:
- Congenital lymphedema—which is apparent at birth or recognized within the first 2 years of life.
- Lymphedema praecox—a disorder that is most often detected at puberty or as late as the third decade of life.
- Lymphedema tarda—which is typically diagnosed in persons older than 35 years.1
Lymphedema tarda—as seen in this patient—is relatively uncommon; it accounts for fewer than 10% of cases of primary lymphedema. A congenital lymphedema, Milroy disease, is inherited as an autosomal dominant disorder. It has been attributed to a mutation that inactivates the vascular endothelial growth factor receptor-3 (VEGFR-3) tyrosine kinase signaling mechanism, which is thought to be specific to lymphatic vessels and plays an important role in lymphatic development.2 Secondary lymphedema is more common than primary disease. Precipitating factors for lymphedema—such as infection, malignancy, radiation, and lymph node dissection—must be ruled out before primary disease can be diagnosed. The goal of treatment is to control limb swelling. A multimodality regimen includes exercise, the use of gradient pressure garments, massage therapy and, for some patients, external pneumatic compression.3-5 Combination ampicillin and sulbactam therapy was given to this patient for superimposed cellulitis; he was referred for reconstructive surgery.