A 38-year-old woman with the diagnosis of Klippel-Trenaunay Syndrome (KTS) presents with severe pain over venous malformations on the left thigh and severe hyperhidrosis of the left leg and foot. She has a history of pulmonary embolism at age 17. Otherwise, she is healthy and compliant with the use of compression garments. The diagnosis of KTS was made soon after birth because of the port wine stains, slightly larger and longer leg and lateral varicose veins of her left leg. Physical examination shows a port wine nevus on the left buttock with varicose veins and soft tissue hypertrophy along the anterolateral aspect of the left leg and thigh. These areas are tender to palpation and leg elevation eases their appearance. Hyperhidrosis behind the knee and dorsum of the foot and in the interdigital spaces is obvious. Limb length discrepancy is 1 cm. The remainder of the exam is unremarkable.
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