A case of exclusive association of pleural effusions and primary lymphedema, without ungual dystrophy is described. This isolated combination is an uncommon clinical condition with only 11 cases reported in the literature. The pathogenesis is characterized by aplasia-hypoplasia of the lymphatic vessels with consequent defective lymphatic drainage. We present a pleural effusion associated with a left lower extremity cellulitis, we argue about the differential diagnosis and treat the patient with thoracocentesis and aspiration with a favourable evolution without recidive.

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