A Rare Case of Chylothorax in a Patient with Schimmelpenning Syndrome

Chylothorax originating in a patient with Schimmelpenning syndrome is rare and poses a problem in diagnosis and treatment. A 22-year-old male was admitted with dyspnea indicative of a large pleural chylous effusion. Besides conservative dietary treatment measures, the chylous effusion was drained (2,000 mL/day). Computed tomography-lymphography after ligation of the thoracic duct and pleurectomy revealed a small collateral flow of chylous fluid toward the chest wall and entering the thorax. Eventually, local radiation therapy with 36 Gy effectively treated the chylothorax. Five months later, an epitheloid angiosarcoma developing from a preexisting cutaneous lesion was detected and treated by surgical resection.