Massive Unilateral Lower Limb Lymphedema in a 42 Year Old Brazilian Man

This 42-year-old Brazilian man of low socioeconomic status came for a clinical evaluation because of malaise, fever (39oC) and shivering associated with pain in the left lower limb. He stated that Fifteen years ago he underwent surgical procedures on the left thigh and leg, which supposedly gave origin to repeated episodes of dermatolymphangioadenitis (DLA) for about ten years. Benzathine penicillin (1.200.000 units at 5-week intervals) contributed to control the frequency of DLA. Relevant epidemiological data included a previous period living with bare feet in Brazilian forest areas, and in other South American regions. His father worked as a collector of latex from the Hevea brasiliensis tree and suffered chronic edema of the legs, allegedly due to tropical filariasis. The patient had no previous cancer or venous thrombosis, nor cardiac, hepatic, renal, or thyroidal disorders. Physical examination revealed an accentuated lymphedema with circumferential measures many times greater in the left lower limb as compared with the uninvolved right extremity (Figure 1A). Testicles and scrotum were normal, and mildly enlarged elastic lymph nodes were palpated in the left groin. The skin was thickened and with a peau d'orange appearance on the calf (Figure 1B). An extensive surgical scar, as well as two very deep skin folds were evident on the affected extremity. It was worthy of note that cobblestone papules, hyperkeratosis, lichenification, mossy and warty changes were absent. The patient was not obese, and the results of laboratory routine tests were unremarkable. Investigation concerning the differential diagnoses of unilateral elephantiasis affecting his lower extremity included thrombophilic tendency, microbiology, histopathology, and serologic studies.