ESSENTIALS OF DIAGNOSIS
Painless persistent edema of one or both lower extremities, primarily in young women.
Pitting edema without ulceration, varicosities, or stasis pigmentation.
There may be episodes of lymphangitis and cellulitis.
When lymphedema is due to congenital developmental abnormalities consisting of hypoplastic or hyperplastic involvement of the proximal or distal lymphatics, it is referred to as the primary form. The obstruction may be in the pelvic or lumbar lymph channels and nodes when the disease is extensive and progressive. The secondary form of lymphedema involves inflammatory or mechanical lymphatic obstruction from trauma, regional lymph node resection or irradiation, or extensive involvement of regional nodes by malignant disease or filariasis. Lymphedema may occur following surgical removal of the lymph nodes in the groin or axillae. Secondary dilation of the lymphatics that occurs in both forms leads to incompetence of the valve system, disrupts the orderly flow along the lymph vessels, and results in progressive stasis of a protein-rich fluid. Episodes of acute and chronic inflammation may be superimposed, with further stasis and secondary fibrosis.
Hypertrophy of the limb results, with markedly thickened and fibrotic skin and subcutaneous tissue (Figure 12–4) in very advanced cases (eFigures 12–18 and 12–19).
Lymphedema of entire left leg. (Reproduced, with permission, from Orkin M, Maibach HI, Dahl MV [editors]. Dermatology. Originally published by Appleton & Lange. Copyright © 1991 by The McGraw-Hill Companies, Inc.)
Mossy foot with lymphatic excrescences. (Reproduced, with permission, from Orkin M, Maibach HI, Dahl MV [editors]. Dermatology. Originally published by Appleton & Lange. Copyright © 1991 by The McGraw-Hill Companies, Inc.)
Lymphedema with a dorsal pedal hump and exaggerated skin folds near the ankle. (Used, with permission, from Dean SM, Satiani B, Abraham WT. Color Atlas and Synopsis of Vascular Diseases. McGraw-Hill, 2014.)
Lymphangiography and radioactive isotope studies may identify focal defects in lymph flow but are of little value in planning therapy. T2–weighted MRI has been used to identify lymphatics and proximal obstructing masses.
Since there is no effective cure for lymphedema, the treatment strategies are designed to control the problem and allow normal activity and function. Most patients can be treated with some of the following measures: (1) The flow of lymph out of the extremity can be aided through intermittent elevation of the extremity, especially during the sleeping hours (foot of bed elevated 15–20 degrees, achieved by placing pillows beneath the mattress); the constant use of graduated elastic compression stockings; and massage toward ...